Neurocutaneous melanosis (NCM) is a rare congenital disorder characterized by the presence of large or multiple congenital melanocytic nevi in association with benign or malignant proliferation of melanocytes in the leptomeninges. NCM is believed to occur as a consequence of an error in morphogenesis in neural ectoderm in the developing embryo. Animal models suggest that aberrant expression of the hepatocyte growth factor/scatter factor (HGF/SF) may be involved in the pathogenesis in the NCM. While the majority of patients with NCM have large congenital melanocytic nevi in a posterior axial distribution, a significant proportion of patients present with multiple smaller nevi in the absence of a single larger lesion. Neurologic manifestations generally occur within the first two years of life, and are often related to increased intracranial pressure. Associated structural anomalies of the CNS have been reported in NCM, particularly the Dandy-Walker complex. The long-term clinical significance of characteristic magnetic resonance findings in neurologically asymptomatic patients is unclear. Approximately half of NCM patients develop CNS melanoma. The prognosis of symptomatic patients remains poor.