Non-ketotic hyperglycemic hemichorea is a rare clinical complication. It is characterized by dance-like movements in the paralyzed limb, non-ketotic hyperglycemia, and imaging manifestations of high signals in the contralateral basal ganglia of the diseased limb. However some patients still start with atypical clinical manifestations, and related reports are few. Hereon, the clinical and imaging characteristics of 3 atypical patients with non-ketotic hyperglycemic hemichorea admitted to the First Affiliated Hospital of Hainan Medical College were summarized and analyzed, so as to improve clinicians' understanding of rare diseases and reduce unnecessary missed diagnosis and misdiagnosis.