We report a case of 30-year-old man presenting chorea in his legs. A brain computed tomography (CT) scan showed bilateral symmetric calcifications in the basal ganglia, thalamus, cerebellum and subcortical white matter. Laboratory studies showed no abnormalities of serum calcium, phosphate, PTH, lactic acid, pyruvic acid and cerebrospinal fluid. Under the diagnosis of Fahr's disease (FD), we treated with quetiapine (75 mg/day), which completely abolished his symptoms and he showed no other side effect. Our experience suggests that quetiapine is well tolerated in FD patients and effectively treats chorea without extrapyramidal movement.