Café-au-Lait Spots: What They Are and When More Than Six Matter
Updated: 3 days ago
Medically reviewed by Dr. Baraa Alnahhal, MD · Last reviewed: October 17, 2026
Quick answer
Café-au-lait spots are flat, light brown to dark brown birthmarks, round or oval, that can grow with age [1]; about 10% of people have at least one [1]. They are benign and cause no symptoms, but six or more spots can point to a genetic condition such as neurofibromatosis type 1 (NF1) [1]. They need no treatment, though laser removal is possible [1].
What the research says
According to PubMed:
Six or more spots in a young child carries a high risk of NF1. In a retrospective study of children with isolated café-au-lait macules, 170 of 419 who had molecular testing (41%) were diagnosed with NF1. Children older than 29 months with fewer than 6 macules (or at least one atypical macule) had a 0.9% risk (95% CI 0%–2.6%), while children younger than 29 months with 6 or more macules had an 80.4% risk (95% CI 74.6%–86.2%). (Ben-Shachar et al., J Am Acad Dermatol, 2017 [7]).
In children with NF1, the spot count climbs quickly in the first two years. Among 63 children with NF1 who first presented with multiple macules, the average count rose from 5.9 at 6 months to 17.5 at 24 months; 38.1% met the six-spot threshold at 6 months and 96.8% by 24 months. (Alexopoulos et al., Eur J Pediatr, 2026 [6]).
The number of spots at birth tracked with NF1 likelihood and plexiform neurofibromas. In 208 patients with café-au-lait macules (147 with confirmed NF1), the likelihood of NF1 rose with the number of macules at birth, reaching up to 95% with 5 or more, and a higher number was linked to more plexiform neurofibromas (p < 0.001). (Santangelo et al., Cancers (Basel), 2025 [8]).
Study details are from PubMed abstracts.
What are café-au-lait spots?
Café-au-lait (CAL) spots are pigmented birthmarks that appear as patches on your skin. They range from light to dark brown and differ in tone from the rest of your skin. The term "café-au-lait" means "coffee with milk" in French, comparing the birthmark's color to the creamy tone of coffee mixed with milk [1] [4].
These birthmarks can be surprisingly large or surprisingly small. They range from a few millimeters to more than 20 centimeters in diameter [1] — bigger than a dinner plate.
CAL spots are present on newborn babies or show up on the skin early during childhood. They can also develop on adults later in life [1].
What do the different types look like?
Café-au-lait spots come in two types identified by their shape and pattern, both named after U.S. coastlines, and multiple coast of California spots warrant a provider visit.
Types of café-au-lait spots
There are two types of café-au-lait spots, identified by their shape and pattern. Both are named after U.S. coastlines.
Type | Border shape | How common | What to know |
Coast of California | Clear, smooth borders on all sides, like the outline of California's coast | Most common [1] | Can be a single spot or multiple spots with clearly defined borders |
Coast of Maine | Rough, jagged border, like the coastal border of Maine | Less common | Less common pattern of CAL spots |
If you have multiple coast of California CAL spots, visit your healthcare provider. Multiple CAL spots could indicate an underlying condition, like neurofibromatosis (NF1) or other genetic syndromes, which affect your skin and nervous system [1].
Who do café-au-lait spots affect?
Café-au-lait spots can affect anyone. The birthmark is congenital, meaning it is present at birth, although it may be hard to see on a newborn baby's skin. The spots become more visible with age, especially after two years [1].
CAL spots are more prevalent among people with darker skin tones [1], but birthmarks can appear on anyone.
Some CAL spots relate to genetic conditions where they are an inherited trait in your family history. To better understand your risk of having a child with a genetic condition, talk to your healthcare provider about genetic testing.
How common are café-au-lait spots?
Café-au-lait spots are common among children. An estimated 10% of the population has at least one CAL spot [1].
More than six CAL spots are present on an estimated 95% of people diagnosed with neurofibromatosis type 1 (NF1) [1].
Fact | Number |
People in the general population with at least one CAL spot | About 10% |
People diagnosed with NF1 who have more than six CAL spots | About 95% |
Do café-au-lait spots harm the body?
Café-au-lait spots are benign (noncancerous) and harmless [1]. They will not affect your child's body.
If your child has six or more CAL spots, visit your healthcare provider because multiple CAL spots can be a sign of an underlying genetic condition.
What do café-au-lait spots look like?
Café-au-lait spots are flat, painless, light to dark brown round or oval spots, usually found on the torso, arms, legs and buttocks [1].
Symptoms and appearance
Café-au-lait spots have a distinct appearance on the skin that sets them apart from other birthmarks.
They are flat spots on the skin, typically on the torso, arms, legs, and buttocks [1]. The color ranges from light brown to dark brown. The shape is round or oval, measuring between 2 millimeters to more than 20 centimeters in diameter [1]. Each spot has a smooth or rigid border around it.
The spots can grow in size and number with age [1]. Most importantly, spots are painless and do not cause itchiness or other symptoms [1].
What causes café-au-lait spots?
An increase in the number of skin cells called melanocytes causes café-au-lait spots. Melanocytes produce the protective pigment that darkens skin, called melanin. The increase happens in the epidermis — the thin outer layer of your skin [1]. The reason for the increase in melanocyte cells is unknown [1].
People diagnosed with neurofibromatosis type 1 (NF1) have CAL spots due to a mutation of the NF1 gene [1] [3]. The NF1 gene prevents cells from growing too quickly [1] [3]. When it mutates, melanocytes produce too much melanin in your skin.
Sometimes the NF1 gene mutation occurs randomly, without being present in a person's family history [1] [3]. Others can inherit this condition from their parents if one parent has a mutated copy of the NF1 gene — a pattern called autosomal dominant inheritance [1] [3].
Like other autosomal dominant conditions, NF1 needs only one changed copy of the NF1 gene to cause the condition, and signs such as café-au-lait spots usually appear in early childhood. Some features, such as neurofibroma tumors, form when the second copy of the NF1 gene also changes in certain cells [1] [3].
What conditions have café-au-lait spots as a symptom?
Multiple café-au-lait spots — six or more — can be a sign of an underlying genetic condition [1].
Condition | What it involves |
Neurofibromatosis type 1 (NF1) | A genetic disorder affecting skin and nerves; about 95% of people with NF1 have more than six CAL spots |
McCune-Albright syndrome | A genetic condition associated with multiple CAL spots [1] |
Noonan syndrome | A genetic syndrome linked to CAL spots [1] |
Watson syndrome | A genetic condition associated with CAL spots [1] |
If you notice your child has six or more café-au-lait spots on their body, visit your child's healthcare provider.
How are café-au-lait spots diagnosed?
After your child is born, your child's healthcare provider will physically examine your baby to make sure they are healthy. During this exam, they will take note of any birthmarks on your child's body, including café-au-lait spots [1], and ask you to keep an eye on how many your child has as they grow older.
Spots might not be entirely visible on your newborn. They might become more visible after your child turns two years old or after sun exposure [1].
How are café-au-lait spots treated?
Café-au-lait spots do not need treatment [1] [4]. They are benign (noncancerous) and do not cause symptoms.
If you want to remove your café-au-lait spots for cosmetic reasons, talk to your healthcare provider about laser treatment. With laser treatment, there are some risks to weigh. The treated area can develop hyperpigmentation (darkening of the skin) or scarring [1]. The spot's pigmentation might not clear completely, and spots could return after being removed [1].
Treatment option | When it applies | What to know |
No treatment | Standard — spots are harmless and cause no symptoms | Nothing is required |
Laser removal | Cosmetic reasons only, by choice | Risks include hyperpigmentation, scarring, incomplete clearing, and spots returning |
What can I expect with café-au-lait spots?
Café-au-lait spots are present from birth and last for life, and they may change size and shape over time or become more noticeable in the sun.
Outlook
Café-au-lait spots are present from birth and will be with your child for their entire life. The spots may change size and shape over time [1] and could become more noticeable when exposed to the sun.
If you see that your child acquires six or more CAL spots on their body, visit your child's healthcare provider to see if the spots could be a sign of an underlying genetic condition.
Can café-au-lait spots be prevented?
Café-au-lait spots cannot be prevented because there is no way to prevent birthmarks, but people with a family history of NF1 can discuss genetic testing.
Prevention
You cannot reduce your risk of café-au-lait spots because there is no way to prevent birthmarks [1].
For people who have a history of genetic conditions like neurofibromatosis type 1 (NF1) running in their family, talk to your healthcare provider about genetic testing [1]. This helps you understand your risk of having a child with the condition before you plan on becoming pregnant.
How do I take care of my child?
Caring for a child with café-au-lait spots means watching for spots that swell, feel lumpy, grow or number more than six, and seeing a provider if they do.
Living with café-au-lait spots
When should I see my healthcare provider?
Visit your healthcare provider if you notice your child's café-au-lait spots appear swollen, bumpy, or lumpy to the touch, or if they increase in size or number [1]. It is common to have a few CAL spots, but six or more spots can be a sign of an underlying condition [1].
Other symptoms of common underlying conditions that cause CAL spots include [1]:
Warning sign | What it means |
Spots swollen, bumpy, or lumpy to the touch | Needs a provider check |
Spots increasing in size or number | Needs a provider check, especially beyond six spots |
Speech and language problems | Possible sign of an underlying condition |
Noncancerous tumors under the skin (neurofibromas) | Common in NF1 |
Freckles under the armpits | Possible sign of an underlying condition |
High blood pressure (hypertension) | Possible sign of an underlying condition |
Bone growth problems (scoliosis) | Possible sign of an underlying condition |
ADHD | Possible sign of an underlying condition |
What questions should I ask my doctor?
Ask whether your child's café-au-lait spots are a sign of a genetic condition, whether you can get a genetic test to assess your own risk of having a child with a genetic condition, and what you should do if you notice your child develops more than six café-au-lait spots.
What other common questions do people ask about café-au-lait spots?
Café-au-lait spots can appear in adulthood, can occur without neurofibromatosis and are not a rash, though more than six spots may signal an underlying condition.
Can café-au-lait spots suddenly appear in adulthood?
Yes. Café-au-lait spots are most often present on a newborn's skin, but it is common for CAL spots to develop on a person's skin later in life [1]. It is normal to have a few CAL spots, but more than six spots may be a sign of an underlying condition. No matter what age, if you notice six or more CAL spots on your body, contact your healthcare provider.
Can you have café-au-lait spots without having neurofibromatosis?
Yes. It is very common for people to have a few café-au-lait spots on their bodies without having an underlying condition like neurofibromatosis type 1 (NF1) [1]. People diagnosed with NF1 usually have more than six café-au-lait spots along with additional symptoms related to their diagnosis.
Are café-au-lait spots the same as a rash?
No. Although they may look like a rash, café-au-lait spots are not the result of an allergic reaction [1]. CAL spots differ from a rash based on their light to dark brown color (whereas rashes are pink to red) and they do not create symptoms like itchiness that you might get from a rash.
Conclusion
For most people, café-au-lait spots are simply harmless birthmarks — a mark of coffee-and-cream brown that shows up at birth or in early childhood and stays for life. They never itch, never hurt, and never require treatment. The number one matters more than the spots themselves: one to a few spots are normal for about 1 in 10 people, while more than six is a reason to check in with a healthcare provider, because it can point to an underlying genetic condition like NF1. Keeping a simple count as your child grows is the easiest way to stay on top of it.
Curious about skin and birthmarks? Explore more plain-language health guides at Rinnit.com.
Frequently asked questions
What are café-au-lait spots?
Café-au-lait spots are flat, pigmented birthmarks that appear as light brown to dark brown patches on the skin — their French name means "coffee with milk."
Are café-au-lait spots dangerous?
No. They are benign (noncancerous), harmless, and cause no pain, itchiness, or other symptoms.
How common are they?
Café-au-lait spots are common — an estimated 10% of the population has at least one café-au-lait spot.
When do they appear?
Usually at birth or early in childhood, though they may not be fully visible until around age two or after sun exposure. They can also develop in adulthood.
Do they go away?
No. They are present from birth and stay for life, though they may change size and shape over time.
Why are they brown?
Café-au-lait spots are caused by an increase in melanocytes — the skin cells that produce melanin, the pigment that darkens skin — in the outer layer of the skin. The reason for the increase is unknown.
What are the two types of café-au-lait spots?
Coast of California spots have clear, smooth borders and are the most common type. Coast of Maine spots have rough, jagged borders and are less common.
How big can they get?
Café-au-lait spots range from a few millimeters to more than 20 centimeters in diameter and can grow with age.
When should I worry about my child's spots?
See a provider if spots appear swollen, bumpy, or lumpy, or if they increase in size or number. Six or more spots can signal an underlying condition.
What does more than six spots mean?
Six or more spots can be a sign of an underlying genetic condition such as neurofibromatosis type 1 (NF1), McCune-Albright syndrome, Noonan syndrome, or Watson syndrome.
What is the link between café-au-lait spots and NF1?
About 95% of people diagnosed with neurofibromatosis type 1 have more than six café-au-lait spots, caused by a mutation of the NF1 gene that makes melanocytes produce too much melanin.
Can you have spots without NF1?
Yes — it is very common to have a few spots without any underlying condition. People with NF1 usually have more than six spots plus other symptoms.
Do café-au-lait spots need treatment?
No — they are harmless and symptom-free. Laser removal is an option for cosmetic reasons, but it carries risks like hyperpigmentation, scarring, incomplete clearing, and recurrence.
Can café-au-lait spots be prevented?
No — there is no way to prevent birthmarks. People with a family history of NF1 can discuss genetic testing before pregnancy to understand their risk.
Are café-au-lait spots a rash?
No. They are not allergic reactions. Unlike rashes (pink to red and itchy), café-au-lait spots are brown and never itch.
Related reading
This article is for general informational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions about a medical condition. Never disregard professional medical advice or delay seeking care because of something you have read here.
References
Cleveland Clinic. Café-au-Lait Spots. Cleveland Clinic. Last updated March 31, 2022. https://my.clevelandclinic.org/health/diseases/22627-cafe-au-lait-spots
Genetic and Rare Diseases Information Center (GARD), National Institutes of Health. Cafe au lait spots, multiple. Genetic and Rare Diseases Information Center. Last updated September 2026. https://rarediseases.info.nih.gov/diseases/3967/cafe-au-lait-spots-multiple
National Library of Medicine. Neurofibromatosis type 1. MedlinePlus Genetics. Last updated July 1, 2020. https://medlineplus.gov/genetics/condition/neurofibromatosis-type-1/
National Library of Medicine. Birthmarks - pigmented. MedlinePlus Medical Encyclopedia. Review date October 13, 2024. https://medlineplus.gov/ency/article/000828.htm
Jha SK, Syed HA. Cafe Au Lait Macules. Updated February 15, 2026. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2026 Jan-. PMID 32491424. https://www.ncbi.nlm.nih.gov/books/NBK557492/
Alexopoulos A, Ntokos D, Kontara L, et al. Early quantitative progression of café-au-lait macules and diagnostic threshold fulfilment by 24 months in neurofibromatosis type 1. Eur J Pediatr. 2026;185(6):431. PMID 42174263. https://doi.org/10.1007/s00431-026-07092-1
Ben-Shachar S, Dubov T, Toledano-Alhadef H, et al. Predicting neurofibromatosis type 1 risk among children with isolated café-au-lait macules. J Am Acad Dermatol. 2017;76(6):1077-1083.e3. PMID 28318682. https://doi.org/10.1016/j.jaad.2017.02.027
Santangelo A, Chelleri C, Tomasino M, et al. Café-au-lait macules in neurofibromatosis type 1: birthmark or biomarker? Cancers (Basel). 2025;17(9):1490. PMID 40361417. https://doi.org/10.3390/cancers17091490
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