Peutz-Jeghers syndrome is a disease manifested by a combination of mucocutaneous pigmentation and gastrointestinal (GI) 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 enteroscopy is presented. The patient had eight large polyps that required three enterotomies for removal. Only three of the polyps were palpable. Two intussusceptions were reduced. The advantages of intraoperative enteroscopy are that 1) it provides accurate assessment of the extent of the disease, 2) smaller polyps can be treated endoscopically, preventing enterotomies, and 3) the endoscopist can direct the surgeon to the appropriate enterotomy sites. A combined surgical and endoscopic approach for the management of Peutz-Jeghers is successful. It more accurately removes the cause of the major morbidity associated with the disease and may allow the patient a longer interval between laparotomies.