Fahr's disease, also known as familial idiopathic basal ganglia calcification or bilateral strio-pallido-dentate calcinosis, is a rare entity characterized by abnormal vascular calcium depositionin the thalamus, basal ganglia, cerebral cortex and the dentate nuclei of the cerebellum. Intracranial ependymomas comprise approximately 2% to 9% of all neuroepithelial tumors. It is reported that supratentorial ependymoma constitute 30% to 50% of all intracranial ependymal tumors. Among supratentorial ependymomas, approximately 50% of them are located extraventricular and demonstrate no relationship with the ventricularsystem.The association of brain tumor with Fahr's disease is a rare entity and has been reported several times before. Whereas, to best our knowledge, the association of Fahr's disease and supratentorial anaplastic ependymoma is described in the present study for the first time.