Background: Chorea hyperglycemia basal ganglia syndrome (CHBS) is a rare disorder accounting for only 1% of the total chorea-ballism cases.It is characterized by an acute or subacute, most often unilateral evolvement of chorea/ballism after nonketotic hyperglycemic episode, MRI characteristic features mainly of T1-hyperintense signal in the basal ganglia, and alleviation of symptoms as glycemic control is achieved.Case presentation: A 76-year-old poorly controlled diabetic female, presented with acute gait disorder.During her admission, she had a nonketotic hyperglycemia episode with blood glucose levels up to 700 mg/dl.A few days later she developed bilateral choreiform limbs' movements more pronounced in her lower limbs.A magnetic resonance imagining (MRI) scan of the brain revealed T1 hyperintense and T2 hypointense signals in the basal ganglia bilaterally suggesting CHBS.A gradual control of her blood sugar was successful, achieving a HbA1C of 7%.During an 18-month follow-up, there was no improvement in her chorea despite strict glycemic control.Conclusions: The emergence of chorea in uncontrolled diabetic patients should raise the suspicion of CHBS in the setting of characteristic T1 MRI hyperintensity in the basal ganglia.Our report adds to the database a case of CHBS with an unfavorable outcome, emphasizing the importance of awareness of this diagnosis and glycemic control.