Abstract: Objective To identify the best therapeutic regimen for a rare nonketotic diabetic patient with epilepsy. Methods We searched the Cochrane Library （Issue 4, 2011）, PubMed （1966 to April 2012）, EMBASE（1974 to April 2012）, CBM （1978 to April 2012） and CNKI （1979 to April 2012） to identify relevant evidences. The quality of the retrieved studies was critically assessed. Results A total of 316 records were retrieved. No clinical guidelines, systematic reviews or clinical randomized studies were identified. According to sixty-five treatment-related literatures involved 3 interventions, combined with the patient preference, small doses insulin was given to control blood sugar and fluid infusion, with improved microcirculation and prevention of cerebral edema, blood sugar levels gradually stabilized, and there was no occurrence of epileptic seizures anymore. Follow-up of 8 months, blood glucose was under control and no more epileptic seizures relapse. Conclusion Early diagnosis of nonketotic diabetes epilepsy could prevent the misdiagnosis and improve clinical outcome.