Peutz-Jeghers syndrome (PJS) is an autosomal dominant hereditary hamartomatous polyposis with predominant localization in the jejunum and ileum and high risk of bowel perforation after traditional polypectomy. The modern enteroscopy is the only possible technique for visualizing and performing intraluminal endoscopic microsurgical manipulations in the deep sections of the small intestine. The study aims to develop an optimal method for the diagnosis and treatment of polyps in children with PJS. During 2015 to 2018 we conducted 30 comprehensive examinations of children with PJS in The Department of Endoscopic Research of the National Medical Research Center for Children's Health. We performed esophagogastroduodenoscopy and colonoscopy with removal of polyps more than 7 mm, then video capsule endoscopy and, guided by this, therapeutic single-balloon enteroscopy. Our technique for removal of polyps is general in all parts: (1) creating a "resistant pillow"; (2) electroexcision of polyp; (3) clipping the removal site. Successfully performed electroexcision of polyps, which were located in the deep parts of the small intestine at a distance of 30 segments (one segment is 10 cm), reached a diameter of 2.5 cm, had a long pedicle. The postoperative period was uneventful. We have developed an optimal method of diagnostic and therapeutic measures, the observance of which allows us to avoid delayed perforations of the small intestine in the area of polypectomy in the postoperative period in children with PJS. Thanks to this technique, modern enteroscopy is becoming the only possible alternative to bowel resection in children with PJS.