Objective To investigate the diagnosis and treatment and follow-up results of primary melanoma of the central nervous system (CNS). Methods A retrospective analysis was performed on the clinical manifestations, radiological features, pathological findings, surgical treatment, and prognosis of 10 patients with primary melanoma of the CNS who were hospitalized from January 2002 to January 2013. Results Of the 10 cases, 8 had intracranial melanoma, 1 had intraspinal melanoma, and 1 had melanoma at the craniocervical junction. Total resection was achieved in 8 cases, and subtotal resection in 2 cases. The pathological examination showed that melanoma was found in 8 cases, and meningeal melanocytoma in 2 cases. During follow-up (3 months to 11 years), 6 cases died, and one of them survived for 7 years after operation with comprehensive treatment and died of complications. The median survival time was 1.1 years. 4 cases showed a significant recovery after surgery and a full course of radiotherapy. Conclusions Primary melanoma of the CNS is an extremely rare tumor and radiological presentation and preoperative diagnosis could be difficult and confusing, leading to a high misdiagnosis rate. The optimal treatment for this disease is surgical resection followed by radiotherapy or chemotherapy for patients whose tumors are not totally removed. Comprehensive treatment for primary melanoma may prolong the survival period. However, the ultimate prognosis remains poor.