Primary intracranial melanoma is a rare and uncommon lesion. Association of primary CNS melanoma in an albino has not been reported in literature searched till now. We are presenting a rare case of primary CNS melanoma in a 52years old male with occulocutaneous albinism. The patient presented with repeated episodes of generalized headache, vomiting and ataxia for duration of 5months. MRI examination showed a tumor in the posterior fossa that was diagnosed as Ependymoma radiologically. Surgical treatment with total removal of tumor was done. Intraoperative squash cytology and frozen section followed by histopathology confirmed the diagnosis of Melanoma. A thorough investigation of the patient was performed including chest radiography, ocular examination, ultrasonography of abdomen and barium enema to rule out any other site of primary melanoma in the body. Thus a final diagnosis of primary CNS melanoma was given.