Peutz–Jeghers syndrome (PJS) is a genetic, autosomal dominant disorder recognized by hamartomatous polyps in the gastrointestinal tract(especially stomach and intestine)and pigmented mucocutaneous lesions. It predisposes agonizes to diverse malignancies (gastrointestinal, pancreatic, breast, uterine, lung, ovarian and testicular tumors). History of intussusceptions, bleeding and obstruction are general complications in patients with PJS. Double balloon enteroscopy (DBE) permits inspections and treatment of the small bowel. Polypectomy applying DBE may obviate the requirement for repeated urgent operations and small bowel resection that guides to short bowel syndrome. Polypectomy and prophylaxisof the complete small bowel is the gold standard in affected patients.