Objective To retrospectively analyze the epidemiology, clinical characteristics and causes of misdiagnosis of Juvenile myoclonic epilepsy (JME) in Xinjiang Uygur Autonomous Region, so as to provide basis for improving the diagnosis and treatment of JME. Methods 979 patients with epilepsy in Xinjiang Uygur Autonomous Region were analyzed retrospectively. There.were515males and 464females,average.age(18.66+8.31)years,.The epidemiological characteristics of JME were analyzed. The clinical characteristics, EEG, treatment effect and prognosis of patients diagnosed with JME were analyzed. The causes of misdiagnosis, missed diagnosis and delayed treatment were analyzed. Results The proportion of JME in 979 patients with epilepsy was 1.4%, a total of 14 cases. The median age of onset was (15+5.83) years, the median time from onset to treatment was 3 years, and the median time from onset to diagnosis was 6 years. All patients showed myoclonic seizures, 13 cases were complicated with generalized tonic clonic seizures, and 4 cases were accompanied by absence seizures. EEG findings include normal background activity, 3-6 Hz generalized spikes or frontal dominant multiple spikes at the beginning of arousal. seven patients were treated with levetiracetam, and the other seven patients were treated with lamotrigine and / or sodium valproate. Incomplete collection of medical history and failure to describe the medical history in detail are the main reasons for delaying diagnosis. Conclusion Juvenile myoclonic epilepsy is an treatable disease, but it is easy to be misdiagnosed. The rate of misdiagnosis and missed diagnosis of JME in Xinjiang is higher, and the delay of diagnosis and treatment is longer. The inquiry of more detailed and demonstrative medical history is of great significance to improve the diagnostic accuracy.