RESUMO
Congenital melanocytic nevi are rare lesions which depending on their size and location may cause major cosmetic and psychological problems. Large congenital melanocytic nevi may undergo malignant change and can also be associated with neurocutaneous melanosis. The different treatment approaches reach different levels of the skin. Complete excision is the treatment of choice, but is not always possible with giant nevi. Superficial treatment can reduce the pigmentation, but repigmentation is not uncommon. Incomplete removal of melanocytic nevi does not reduce the melanoma risk. Surgical intervention must be carefully planned; the advantages and disadvantages of the different modalities must be discussed with the parents. The risk of malignant transformation must be weighed up against the expected aesthetic and functional outcomes.