Fahr's disease is a rare entity characterized by abnormal calcification in the bilateral basal ganglion regions and cerebellum. Concurrence of Fahr's disease with brain tumor is associated with an even lower incidence; the present study describes the fifth such case, in which a 32-year-old female presented with a 1-month history of headache and unsteady walking. Pathology and imaging studies resulted in a diagnosis of low-grade astrocytoma with calcified deposition. The patient recovered uneventfully. Following a review of the literature, several similarities among the reported cases were revealed. Patients were relatively young at the onset of the disease. The pathological diagnosis for all brain tumors was astrocytoma. The cerebellum was the predominant location of the brain tumor. The imaging studies of these cerebral astrocytomas were almost identical, with a cystic glioma occurring adjacent to a neighboring calcified dentate nucleus. Furthermore, the development of astrocytoma in the bilateral cerebellar hemisphere was not uncommon, which warrants close follow-up for these patients.