Congenital melanocytic nevi are benign skin tumours with a population-based
prevalence of 1-6%. They run an increased life-time risk of transformation
into malignant melanoma. Additionally, they can be associated with an invo
lvement of the leptomeninges (neurocutaneous melanosis), and may cause cons
iderable cosmetic and psychic problems. In contrast to the past, the risk o
f congenital melanocytic nevi developing into melanoma is now regarded as w
ell-established. Thus, excision is recommended whenever possible. Large les
ions which do not allow complete excision should be treated by dermabrasion
within the first weeks of life.