We report on a very rare case of dorsal intramedullary melanocytoma in a 62-year-old man. The tumor was resected and gross total removal was achieved. During a 2-year follow-up period in which no radiotherapy was given, the tumor did not recur. We review the literature on these tumors and present disease criteria to distinguish melanocytoma from primary malignant melanoma. Patients and investigators should be cautioned that an intramedullary melanocytoma may recur and give rise to metastatic spread via the cerebrospinal fluid.