Abstract
Neurocutaneous melanosis (NCM) associated with Dandy-Walker malformation is a very rare congenital neurodysplasia with the same origin. Primary intracranial melanocytic and dermoid tumors are also benign congenital lesions that usually arise from the leptomeninges and are formed by the inclusion of cutaneous ectoderm at the time of neural tube closure. The authors describe a patient with coexisting intracranial meningeal melanocytoma, NCM with Dandy-Walker malformation, and intraventricular dermoid tumor.
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