Objective To analyze the clinical manifestations and brain CT and MRI imaging features of non-ketotic hyperglycemia hemichorea, aiming to improve the understanding of the disease and avoid misdiagnosis and missed diagnosis. Methods Clinical data and CT and MRI imaging findings of 6 patients with clinically-proved non-ketotic hyperglycemia hemichorea from March 2016 to February 2018 in Xingning People's Hospital were analyzed retrospectively. Six patients underwent unenhanced CT scan, unenhanced MRI scan and enhancement, and DWI sequence(6), SWI sequence scan(3), follow-up unenhanced CT scan(3). Location, size, morphology, density/signal characteristics and degree of enhancement of the lesion were observed and analyzed. Results Six patients included 2 males and 4 females. Six patients showed clinical manifestations of unilateral limb or facial involuntary, irregular dance-like movements. Blood glucose was 22.56- 33.42 mmol/L at the onset. Urine ketones were all normal. Patchy hyperdense lesion in the caudate nucleus or lenticular nucleus in the contralateral basal ganglia of the affected limb were seen on CT and T1WI of 6 patients, while low signals on T2WI and FLAIR. DWI showed a low signal, of which 2 cases showed slightly higher or lower signals. SWI is hypo or isointense. The lesions showed mild or marked enhancement. After treatment with hypoglycemic and other treatments, the clinical symptoms of the patients were alleviated to varying degrees, and the imaging findings were also changed. One case was reexamined after 5 hours of treatment, and the lesions disappeared. The remaining 2 cases were treated for half a month and 1 month , follow-up CT scan showed that the lesion range was smaller than before and the density was lower than before. Conclusion Nonketotic hyperglycemia hemichorea cerebral CT and MRI have typical imaging findings, combined with medical history and clinical symptoms and signs, can make accurate diagnosis in time to avoid misdiagnosis and missed diagnosis.