These flat, light brown patches — the color of coffee with milk, hence the name — are caused by a localized increase in melanin production. They have smooth, well-defined borders and uniform color. They can be present at birth or appear in the first few years of life.
One or two cafe-au-lait spots are extremely common and completely harmless. Up to 25% of healthy children have at least one. No workup needed, no monitoring needed, no referral needed.
Six or more is a different situation. Having six or more cafe-au-lait spots larger than 5mm (in children before puberty) is one of the diagnostic criteria for neurofibromatosis type 1 (NF1), a genetic condition that affects about 1 in 3,000 people. NF1 causes benign tumors to grow along nerves and can affect the skin, bones, eyes, and nervous system.
To be clear: cafe-au-lait spots alone do not diagnose NF1. The condition has multiple diagnostic criteria, and meeting just one (like the spots) triggers an evaluation, not a diagnosis. Many children with multiple cafe-au-lait spots turn out not to have NF1. But the spots are often the earliest visible sign, which is why pediatricians count them.
What to do: Count your baby's cafe-au-lait spots at each well-child visit. If the count reaches six or more spots that are each larger than 5mm, mention it to your pediatrician. They will examine for other NF1 features (freckling in the armpits or groin, Lisch nodules in the eyes) and may refer to genetics if warranted.