A 63-year-old man with right hemiparesis was found (on MRI) to have an expansive intramedullary tumorous lesion at the C2-C3 level. After complete neurosurgical tumor resection, the tumor was histologically categorized as an intermediate grade of intramedullary melanocytoma, an uncommon neoplasm. Based on this peculiar case and review of the literature, radical surgical resection appears to be the therapy of choice for intramedullary melanocytomas. However, their high recurrence rate and aggressive behavior suggest the need for close followup with serial MRI.