Objective　To investigate the clinical manifestation,electroencephalography characteristics and prognosis of myoclonic absence epilepsy (MAE).Methods　Data from 14 patients diagnosed as MAE in the First Affiliated Hospital of Harbin Medical University from 2013 to 2019 were collected and analyzed.Results　Among the 14 cases,8 were male and 6 were female.Of all the 14 cases,myoclonic absences (MA) was the sole or main seizure symptoms,an abrupt onset and termination,a high frequency at least several times to nearly 100 times per day.They can be elicited by hyperventilation and intermittent light stimulation.The ictal EEG shows bilateral,synchronous and symmetrical spike and wave discharges repeated at 3 Hz in strict relation with myoclonias recorded on EMG.The effective drug was valproate,or combined with other antiepileptic drugs.The ages at follow up ranged from 5 years to 15 years.Seizures were controlled for 4 months to 3 years in 8 cases.Conclusion　MAE is a rare type of children with epilepsy syndrome characterized by MA.The diagnosis of MAE depends on the clinical symptoms and V-EEG findings.Early and exact diagnosis contributes to the prognosis of MAE.