The clinical, radiological and autopsy features of a 5-month-old child with neurocutaneous melanosis are described. The patient had multiple disseminated benign cutaneous nevi. The pneumoencephalogram showed non-obstructive hydrocephalus. The patient died at the third day of hospitalization and autopsy was performed. A mild hydrocephalus and melanic specks in cerebellum and in the brainstem were disclosed.