The incidence of primary central nervous system (CNS) melanocytic neoplasms is relatively low comparing to systemic ones. The performance of the tumor is variable. With poor clinical experience, the diagnosis and treatment of such tumors present to be a challenge. Amelanotic melanoma is an especially rare subtype. Only several cases have been reported.We report a case of intracranial amelanotic melanoma. Preoperative assessment revealed progressive right frontal mass. The patient underwent tumor resection. The pathologic analysis reported amelanotic melanoma of intermediate grade. The further examination of the whole brain and body was negative. The familial history was also negative. The patient recovered uneventfully and went on for radiotherapy and chemotherapy. After a follow-up period of 5 months, the patient was tumor-free.This is the second report about primary CNS amelanotic melanoma. We summarized characteristics of the primary CNS melanocytic lesions and amelanotic melanoma with review of the literature and review of cases of our department.