Peutz-Jeghers syndrome is a rare disease manifested by a combination of mucocutaneous pigmentation and gastrointestinal polyposis. The major morbidity results from intussusception, obstruction, and bleeding. Standard surgical management has been to perform enterotomies at the site of palpable polyps. A method of treating Peutz-Jeghers syndrome surgically with combined intraoperative enteroscopic polypectomy, is herein presented. The patient had multiple small bowel polyps, one of which was very large and required surgical resection. Segmental resection and multiple enterotomies were performed to remove the polyps. During the course of the operation, endoscope (CF-200I, 130 cm) was inserted per enterotomy site and multiple small bowel polyps were removed endoscopically using a snare. The combined surgical and endoscopic approach for the Peutz- Jeghers syndrome was successful. The procedure removes more accurately, the cause of the major morbidity associated with the disease, and may allow the patient a longer interval between laparotomies.