Background::Surgery is a conventional mature treatment for moyamoya disease (MMD). However, whether surgery is also an effective therapy for epileptic type MMD has seldom been investigated systematically. The study aims to summarize the pooled postoperative incidence of seizure and cerebral infarction in pediatric patients with epileptic type moyamoya disease.Method::The study was a systematic review and critical appraisal with a meta-analysis of cohort studies, both prospective and retrospective. Studies were identified by a computerized search of PubMed, Embase, Web of Science, Wanfang, and CNKI databases. In a literature search, a total of 7 cohort studies were identified. The n I2statistic was used to quantify heterogeneity. A fixed-effect model was used to synthesize the results. The linear regression test of funnel plot asymmetry was used to estimate the potential publication bias.n Results::The pooled estimated postoperative incidence of seizure in pediatric patients with epileptic type moyamoya disease was 23.44%. The pooled estimated postoperative incidence of cerebral infarction in pediatric patients with epileptic type moyamoya disease was 9.12%. Low substantial heterogeneity and potential publication bias were present.Conclusions::Evidence from this study suggests that the postoperative incidence of seizure and cerebral infarction is relatively low. Surgery is an effective and secure therapy for pediatric patients with epileptic type moyamoya disease.“,”Background::Surgery is a conventional mature treatment for moyamoya disease (MMD). However, whether surgery is also an effective therapy for epileptic type MMD has seldom been investigated systematically. The study aims to summarize the pooled postoperative incidence of seizure and cerebral infarction in pediatric patients with epileptic type moyamoya disease.Method::The study was a systematic review and critical appraisal with a meta-analysis of cohort studies, both prospective and retrospective. Studies were identified by a computerized search of PubMed, Embase, Web of Science, Wanfang, and CNKI databases. In a literature search, a total of 7 cohort studies were identified. The n I2statistic was used to quantify heterogeneity. A fixed-effect model was used to synthesize the results. The linear regression test of funnel plot asymmetry was used to estimate the potential publication bias.n Results::The pooled estimated postoperative incidence of seizure in pediatric patients with epileptic type moyamoya disease was 23.44%. The pooled estimated postoperative incidence of cerebral infarction in pediatric patients with epileptic type moyamoya disease was 9.12%. Low substantial heterogeneity and potential publication bias were present.Conclusions::Evidence from this study suggests that the postoperative incidence of seizure and cerebral infarction is relatively low. Surgery is an effective and secure therapy for pediatric patients with epileptic type moyamoya disease.