The syndrome of acute bilateral basal ganglia lesions associated with uremia presents with parkinsonism, altered mental status, and chorea in association with specific imaging findings in the basal ganglia. It is an uncommon syndrome seen generally in patients with diabetes mellitus and renal failure. We report a male patient with diabetes mellitus who received hemodialysis treatment 3 days a week for 5 years and suffered from choreic movements developed suddenly and associated with bilateral basal ganglia lesions. In the brain magnetic resonance (MR) imaging, isointense was detected in sequence T1 in the bilateral basal ganglions and hyperintense lesion was determined in T2 and FLAIR sequences. The patient was administered daily hemodialysis and neuroleptic treatment. After intensified hemodialysis, his symptoms and follow-up brain MR imaging showed marked improvement. The underlying mechanism of such lesions may be associated with metabolic, as well as vascular factors. Acute choreic movements may be seen in patients with diabetic nephropathy and intensification of hemodialysis treatment along with blood glucose regulation may provide improvement in this syndrome.