Several cases of bilateral basal ganglia lesions seen in magnetic resonance imaging initiated a review of the anatomy, pathology, and differential diagnoses of this region. There are a variety of disease entities that present as symmetrical basal ganglia abnormalities. Although these findings may not indicate a specific diagnosis, knowledge of the characteristics of diseases that affect this area can limit the differential considerations. Clinical information is often essential for narrowing the possible pathology that can be found here. The purpose of this article is to review the anatomy of the basal ganglia, the pathologies, clinical histories, and imaging characteristics that can cause bilateral basal ganglia lesions.