Background Several studies have investigated the role of PAI-1 4G/5G and ACE I/D polymorphisms in the etiology of pediatric sepsis, but the results are inconsistent. We performed a meta-analysis to assess for any associations. Methods: A comprehensive literature search on PubMed, web of science, and CNKI database was conducted up to April 15, 2020. Results: There were twelve case-control studies involving seven studies with 860 cases and 1144 controls on PA-1 4G/5G and five studies with 1602 cases and 1585 controls on ACE I/D. PAI-1 4G/5G and ACE I/D polymorphisms were associated with an increased risk of pediatric sepsis in the global population. Stratified analysis by ethnicity showed a significant association in the Caucasians children. Conclusions: The meta-analysis suggests that the PAI-1 4G/5G and ACE I/D polymorphisms may be risk factors for development of pediatric sepsis in the global population. [ABSTRACT FROM AUTHOR]