OBJECTIVE To investigate the clinical features of malignant melanoma (MM) in the central nervous system (CNS) and to improve the diagnosis and treatment of this disease. METHODS Seven MM-in-CNS patients' records between September 1996 and April 2007 were analyzed retrospectively, including 6 males and 1 female aged 18-74 years. The 5 cases were located in the supra-tentorial area, 1 in the spinal cord and 1 in the whole brain. CT or MRI scan was applied. The lesion was in the right frontal area in 4 cases, in the right temporal are in 1 case, in the left temporal area in 1 case, in the left apex area in 1 case and in the cervical spinal cord of C5-7 in 1 case. Six patients underwent neurosurgical operation and 1 patient received the Gamma Knife therapy. The pathological examination revealed that 2 cases were metastatic MM and 5 were primary. RESULTS One patient with primary MM received no follow-up, and the rest 6 patients were followed up for 2 weeks to 2 years with the time of median 8 months. One patient with metastatic MM died 2 months after operation, 1 patient to with metastatic MM died 2 weeks after Gamma-Knife treatment, 1 patient with metastatic MM with primary MM died 2 years after operation, and 3 patients with primary MM were still alive and self-independent 6, 10 and 24 months after operation, respectively. CONCLUSIONS Since MM-in-CNS is short of specificity in clinical symptoms and signs, its diagnosis mainly relies on the pathological examination and is assisted by MRI. The combined therapy giving priority to operation is recommended.