Background: Melanocytomas are rare pigmented tumors of the central nervous system (CNS). They are more frequent in females in their fifties and are typically benign, but locally aggressive lesions that very are rarely found in the spine. They can occur in all the age groups. Case Description: A 62-year-old male presented with acute paraplegia a attributed to a C7-T1 intradural extramedullary melanocytoma. Urgent surgery, consisting of subtotal removal of the lesion, resulted in subtotal recovery of motor function with full resolution of his sensory deficit. Histologically, the lesion proved to be a melanocytoma. Conclusion: Rarely, patients with intradural extramedullary melanocytomas of the spine may present with acute paralytic deficits warranting emergent surgical intervention.
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