Objective To investigate the clinical features and prognosis of PRRT2-related paroxysmal disorders. Methods Retrospected clinical data of one cases of benign infantile epilepsy diagnosed by gene diagnosis. Results The clinical manifestations of this Children were recurrent convulsions,cluster seizures,and no obvious abnormalities in physical examination. Genetic examination showed mutation of PRRT2 frameshift. Her mother had a history of paroxysmal motor-induced dyskinesia,and her uncle and cousin had a history of convulsions when they were young. The patient did not convulse after oral oxcarbazepine. Conclusions PRRT2 gene-related paroxysmal diseases are characterized by self-limited infantile epilepsy and paroxysmal motor-induced dyskinesia. Genetic diagnosis is needed for definitive diagnosis.The main treatment of this disease is to control epilepsy,and the prognosis is good.