Hemichorea is a disorder characterized by abnormal, continuous, nonrhythmic, jerky, and distal movement involving one side of the body. It may result from cerebrovascular insult to basal ganglia, or from other causes including neoplasm, infection, and non-ketotic hyperglycemia. We report the clinical, laboratory, and neuroimaging data with treatment response of a Saudi woman who has diabetes with left side hemichorea, involving the face, and upper and lower extremities, with unilateral right striatal hyperintense signal changes in T1 weighted MRI, and a hyperglycemic state of longstanding uncontrolled diabetes. Literature review suggested a syndrome with a triad of symptoms: non-ketotic hyperglycemia, hemichorea, and T1 MRI striatal hyperintensities. As the number of internationally reported cases is still modest, reporting more patients will highlight aspects pertaining to the diagnosis and treatment of this condition. We present a patient who had a sustained therapeutic result from haloperidol and clonazepam.