Objective To investigate the clinical features, electroencephalographic (EEG) characteristics, and treatment of perioral myoclonia with absences (POMA). Methods The clinical features, EEG characteristics, and treatment in two pediatric patients with POMA were retrospectively analyzed. The related literatures were reviewed. Results In two pediatric patients POMA were onset with febrile convulsion. The clinical features were that perioral muscles were contracted rhythmically accompanied by absence and disturbance of consciousness. The episodes were frequent, each lasting a few to 10 seconds. The EEG characteristics during attack phase were generalized discharges of 3 Hz spikes and wave burst, while during interictal phase they were medium-high amplitude spine-slow waves, slow wave dissemination or short array. Brain MRIs were normal. One patient had slightly delayed mental and motor development. One patient had a family history of epilepsy. Both of them were treated with sodium valproate and the seizure was controlled. However, one patient relapsed after drug withdrawal. Conclusions POMA is an idiopathic comprehensive epilepsy syndrome featured by perioral myoclonus with absence. The video-EEG is of great value in diagnosis.