ObjectiveTo systematically review quality of evidence-based pneumocystosis guidelines for children, compare the differences and similarities among recommendations, and to provide references for clinical application. MethodsDatabases such as TRIP, PubMed, CNKI, VIP, WanFang Data, CBM, U.S National Guideline Clearinghouse (NGC), and Guidelines International Network (GIN) were searched to collect evidence-based guidelines on medication therapy for children with pneumocystosis. Methodological quality of included guidelines was evaluated according to the AGREE Ⅲ instrument, and the differences and similarities among recommendations were compared. ResultsA total of 3 evidence-based guidelines concerning children with pneumocystosis were included, of which, 2 were made by the USA and 1 by international academic organizations. Only 1 guideline was especially suitable for children, while others were for children of different ages and adults. The results of AGREE Ⅲ scoring showed that, among 6 domains which were rated using the AGREE Ⅲ instrument, "scope and purpose", "stakeholder involvement", "rigor of development", "clarity and presentation" and "editorial independence" were scored more than 60%; while "applicability" was only scored 46%, 25% and 31%. Besides, the grading of evidence and grading of recommendations were different. The medication recommendations of different guidelines were not the same. ConclusionThe quality of 3 guidelines is not high, and the grading of evidence and the strength of recommendation are still needed to be unified. The target population and the recommendations in the guidelines are different.