Fahr’s disease is a rare disorder characterized by bilateral and symmetric calcifications in brain structures, including the basal ganglia, cerebellum, thalamus, and cerebral white matter. The findings of 123 I-Ioflupane SPECT in Fahr’s disease have only been reported in a limited number of case reports. We present the case of a 64-year-old woman with Fahr’s disease who had reduced uptake in the striatum on both visual and quantitative assessment of 123 I-Ioflupane SPECT images. The striatal involvement was asymmetric, despite the presence of substantially symmetric calcifications on CT and MRI.