This systematic review aims to summarize the body of available literature on pediatric extracorporeal cardiopulmonary resuscitation in order to delineate current utilization, practices, and outcomes, while highlighting gaps in current knowledge. PubMed, Embase, Scopus, Cochrane Library, and ClinicalTrials.gov databases. We searched for peer-reviewed original research publications on pediatric extracorporeal cardiopulmonary resuscitation (patients < 18 yr old) and were inclusive of all publication years. Our systematic review used the structured Preferred Reporting Items for Systematic Reviews and Meta-Analyses methodology. Our initial literature search was performed on February 11, 2019, with an updated search performed on August 28, 2019. Three physician reviewers independently assessed the retrieved studies to determine inclusion in the systematic review synthesis. Using selected search terms, a total of 4,095 publications were retrieved, of which 96 were included in the final synthesis. Risk of bias in included studies was assessed using the Risk of Bias in Non-Randomized Studies of Interventions-I tool. There were no randomized controlled trials of extracorporeal cardiopulmonary resuscitation use in pediatrics. A vast majority of pediatric extracorporeal cardiopulmonary resuscitation publications were single-center retrospective studies reporting outcomes after in-hospital cardiac arrest. Most pediatric extracorporeal cardiopulmonary resuscitation use in published literature is in cardiac patients. Survival to hospital discharge after extracorporeal cardiopulmonary resuscitation for pediatric in-hospital cardiac arrest ranged from 8% to 80% in included studies, and there was an association with improved outcomes in cardiac patients. Thirty-one studies reported neurologic outcomes after extracorporeal cardiopulmonary resuscitation, of which only six were prospective follow-up studies. We summarize the available literature on: determination of candidacy, timing of activation of extracorporeal cardiopulmonary resuscitation, staffing/logistics, cannulation strategies, outcomes, and the use of simulation for training. This review highlights gaps in our understanding of best practices for pediatric extracorporeal cardiopulmonary resuscitation. We summarize current studies available and provide a framework for the development of future studies.