[Giant pigmented nevus associated with leptomeningeal melanocytosis]

A case of a thirteen-year old boy is reported with congenital pigmented nevi on the large part of the trunk and on the extremities. From his age of six generalised epileptiform convulsions were present. Owing to the suspicion of intracranial process he was examined with a negative result. He died of brain edema. On section cerebrospinal leptomeningeal melanocytosis was found beside the nevi. In addition to the clinical findings and treatment the morphological lesions are detailed too at macroscopic, microscopic and ultrastructural level discussing the possibility of the malignisation.