BackgroundIncreasing numbers of children and adolescents experiencing gender dysphoria/incongruence are being referred to specialist gender services. There are various guidelines outlining approaches to the clinical care of these children and adolescents.AimTo examine the quality and development of published guidelines or clinical guidance containing recommendations for managing gender dysphoria/incongruence in children and/or adolescents (age 0-18). A separate paper reports the synthesis of guideline recommendations.MethodsA systematic review and narrative synthesis. Databases (Medline, Embase, CINAHL, PsycINFO, Web of Science) were searched to April 2022 and web-based searches and contact with international experts continued to December 2022, with results assessed independently by two reviewers. The Appraisal of Guidelines for Research and Evaluation tool was used to examine guideline quality.ResultsTwenty-three guidelines/clinical guidance publications (1998–2022) were identified (4 international, 3 regional and 16 national). The quality and methods reporting in these varied considerably. Few guidelines systematically reviewed empirical evidence, and links between evidence and recommendations were often unclear. Although most consulted with relevant stakeholders, including 10 which involved service users or user representatives, it was often unclear how this influenced recommendations and only two reported including children/adolescents and/or parents. Guidelines also lacked clarity about implementation. Two international guidelines (World Professional Association for Transgender Health and Endocrine Society) formed the basis for most other guidance, influencing their development and recommendations.ConclusionsMost clinical guidance for managing children/adolescents experiencing gender dysphoria/incongruence lacks an independent and evidence-based approach and information about how recommendations were developed. This should be considered when using these to inform service development and clinical practice.PROSPERO registration numberCRD42021289659.