Neurocutaneous melanosis or neurocutaneous melanocytosis is a rare sporadic congenital disorder characterized by the presence of giant and/or multiple satellite congenital melanocytic nevi in the skin and benign melanocytic pigmentation of the leptomeninges. These two defining features were recognized more than a century ago. A third characteristic feature is proliferative nodules arising from giant nevi. The etiology is unknown, but neurocutaneous melanosis is considered a developmental disorder of melanocyte precursors from neural crest. The distinctive unique distribution of the congenital giant nevi that gives a "garment" appearance is also an expression of the neural crest. The neurological manifestations often appear in infancy.The special association of neurocutaneous melanosis with Dandy-Walker malformation complex may be explained by a common pathogenesis. Mortality in infancy and childhood is high. Copyright © 2013 Elsevier B.V. All rights reserved.