Melanoma is a malignant neoplasm of melanin-producing cells. Melanoma usually occurs in the skin, but can also arise in any anatomical site that contains melanocytes, such as mucous membranes, the eyes, and the central nervous system (CNS). Primary CNS malignant melanoma most often develops in the leptomeninges. We report a case of a rare intramedullary melanoma of the thoracic spinal cord. A 78-year-old man was treated with surgery, radiotherapy, and immunotherapy for leptomeningeal spread. We also discuss the role of imaging methods in diagnosis and follow-up. Medullary melanoma occurs more frequently in adults. The most common presenting symptoms are the insidious onset of lower extremity weakness and paresthesia. Magnetic resonance imaging is the method of choice for evaluation. Although there are no imaging features to accurately distinguish primary malignant melanoma from other melanocytic or hemorrhagic tumors, hyperintensity on T1-weighted magnetic resonance imaging should lead to inclusion of this neoplasm in differential diagnosis of spinal cord tumors. Positron emission tomography-computed tomography is a useful auxiliary examination to evaluate the extent of local and metastatic disease. Surgical resection is the primary treatment for intramedullary melanoma. However, the efficacy of adjunctive radiotherapy and chemotherapy for primary spinal cord malignant melanoma is still controversial.