Purpose of Review <p>This review highlights current challenges and advances in the management of pediatric polyposis syndromes with a focus on diagnosis, surveillance, and emerging therapies. This scoping review explores how developments in genetics, endoscopy, and pharmacology are reshaping the care of patients with familial adenomatous polyposis (FAP), Peutz-Jeghers syndrome (PJS), and juvenile polyposis syndrome (JPS).</p> Recent Findings <p>Early diagnosis remains difficult due to nonspecific symptoms and overlapping presentation with other pediatric gastrointestinal disorders. Expanded use of multigene panels has improved genetic identification of implicated variants for pediatric polyposis syndromes. Surveillance strategies are largely based on adult data with gaps in pediatric-specific guidance. Endoscopic management is challenging in patients with a high polyp burden or small bowel disease. Non-steroidal anti-inflammatory drugs have shown chemopreventive effects in FAP, and mammalian target of rapamycin (mTOR) inhibitors show promise for hamartomatous polyps, though pediatric studies are limited.</p> Summary <p>Early recognition, appropriate genetic testing, and tailored surveillance are critical to managing pediatric polyposis syndromes. While endoscopy remains central to care, new therapies may reduce polyp burden and delay the need for surgery. Future research should prioritize pediatric-focused trials and longitudinal registries to guide personalized, evidence-based care.</p>