OBJECTIVES: Patients with Peutz-Jeghers syndrome (PJS) require continuous medical management throughout their lives. However, few case series regarding the clinical course, polyp surveillance, and treatment, including endoscopic ischemic polypectomy (EIP) for pediatric patients with PJS, were reported. We analyzed the current status and clinical course of pediatric patients with PJS under the management of our institute, including those treated with EIP. METHODS: Medical information on double-balloon enteroscopy (DBE) performed between January 2006 and December 2023 and patient backgrounds were retrospectively collected. The location of polyps, breakdown of treatment methods, and differences in complication rates of each treatment method were analyzed. RESULTS: The median age at diagnosis of PJS was 9 years (0-18 years), and the prevalence of intussusception before the first DBE among the patients was 68.2%. In total, 115 procedures were performed in 22 pediatric patients with PJS. There were 100 therapeutic procedures, and the total number of treated polyps was 462 (362, 54, and 46 in the small bowel, colon, and stomach, respectively). Conventional polypectomy was performed for 106 polyps, and ischemic polypectomy was performed for 356 polyps. The incidence rates of post-polypectomy bleeding and perforation associated with conventional polypectomy and EIP were 2.83% and 0.28%, respectively (p = 0.042). Eight patients (36.4%) had polyps larger than 15 mm under the age of 8 years. CONCLUSIONS: Proper imaging evaluation and endoscopic treatment for gastrointestinal (GI) polyps are essential to prevent GI complications in pediatric patients with PJS, even those younger than 8 years old. Moreover, EIP may be the ideal procedure for managing polyps in this population.