The European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) Polyposis Working Group developed recommendations to assist clinicians and health care providers with appropriate management of patients with juvenile polyposis. This is the first juvenile polyposis Position Paper published by ESPGHAN with invited experts. Many of the published studies were descriptive and/or retrospective in nature, consequently after incorporating a modified version of the GRADE system many of the recommendationsare based on expert opinion. This ESPGHAN Position Paper provides a guide for diagnosis, assessment and management of Juvenile polyposis syndrome (JPS) in children and adolescents, and will be helpful in the appropriate management and timing of procedures in children and adolescents. The formation of international collaboration and consortia is proposed in order to monitor patients prospectively to advance our understanding of juvenile polyposis conditions.