Neurocutaneous Melanosis Is Not Always a Benign Disease

Neurocutaneous melanosis is a rare form of neurocutaneous syndrome characterized by the presence of congenital melanocytic nevi in the skin as well as proliferation of the melanocytes in the leptomeninges and the brain. A 22-month-old girl was brought to the outpatient clinic of Mansoura University Children's Hospital, Egypt, with multiple hairy pigmented nevi since birth which progressively increased in size and number over time.We concluded that though neurocutaneous melanosis is a well-known disease, it is important for dermatologists and pediatricians to remember the risk of a potentially fatal association of giant congenital melanocytic nevi.