INTRODUCTION: Transition of care (TOC) is a process that must be planned and executed in a coordinated manner factoring patient, family, disease, and healthcare system. Among youth with gastrointestinal hereditary polyposis syndromes (HPS), poorly planned TOC can have devastating consequences from poor engagement in necessary medical care. This results in increased risk of cancer and related mortality. This review aims to emphasize unique aspects of HPS relevant to TOC, related barriers/challenges, and outline current best practice recommendations. AREAS COVERED: A review was undertaken of transition-focused practice guidelines among youth with chronic conditions, as well as literature from major pediatric gastroenterology societies on the ideal approach for managing polyposis syndromes. Literature from PubMed and Medline, including conference abstracts and proceedings, was reviewed and in the absence of empirically supported evidence, recommendations reflect the opinion of the author experts involved in the care of adolescents and young adults (AYA) with HPS. EXPERT OPINION: Effective TOC requires a structured, patient-centered, individualized process that includes early engagement, incremental education, and multidisciplinary collaboration. Given the unique aspects of HPS, including complex psychosocial and medical needs, there is urgent need for research toward evidence-based strategies enhancing continuity, and mitigating socio-cultural and financial barriers to care.