Neurokutane Melanose mit malignem meningealem Melanom

A male patient with a giant melanocytic nevus and multiple small ones is reported, who revealed a single episode of focal seizure at the age of 6 years. Thereafter, he remained free of any symptoms and signs until the age of 12 years when he developed the full spectrum of neurological manifestations of neurocutaneous melanosis. He died from a malignant meningeal melanoma at the age of 14 years. In view of the severity of the neurological manifestations and the poor prognosis of neurocutaneous melanosis, close monitoring of patients with giant and/or multiple congenital melanocytic nevi is of essential importance for the prompt diagnosis and management of this lethal syndrome.