This report documents the neuropathological findings of astrocytic proliferation and astrocytoma in a patient with Fahr's disease. At autopsy, there was extensive bilateral symmetrical calcification involving basal ganglia, sulcal depth of the cerebral cortex, and dentate nuclei of the cerebellum. A large low-grade astrocytoma was identified in the left parietal lobe. Astrocytic proliferation was also noted in the areas of early calcification and at the margins of large calcareous deposits, away from the tumor.