Neurocutaneous melanosis (NCM) is a rare congenital neurocutaneous syndrome characterized by large or multiple congenital melanocytic nevi and benign or malignant melanocytic tumors of the leptomeninges. The authors report the case of a 5-month-old girl with congenital giant melanocytic nevi who presented with symptomatic hydrocephalus. A right frontal ventriculostomy was performed in the patie...

Neurocutaneous melanosis (NCM) is a rare congenital disease that is characterized by the presence of large or multiple congenital melanocytic nevi and melanotic lesions of the central nervous system. We report here on the CT and MR imaging findings of an unusual case of NCM that was associated with intraventricular dermoid and Dandy-Walker malformation.

The neurofibromatoses are members of the group of neurocutaneous disorders which also include tuberous sclerosis, neurocutaneous melanosis, hypomelanosis of Ito, incontinentia pigmenti, Sturge-Weber syndrome and von Hippel-Lindau disease. Collectively, these conditions have also been referred to as "phakomatoses," a term is derived from the Greek phakos, meaning a lentil or birthmark. All but S...