Polyps occur throughout the gastrointestinal tract in Peutz-Jeghers syndrome, but the most serious problems are encountered in the management of small bowel polyposis. We report here on a case of Peutz-Jeghers syndrome admitted to hospital for intestinal obstruction and anaemia. The patient was submitted to colonoscopy, oesophagogastro-duodenoscopy and small bowel enema. At laparotomy, multiple intussusceptions were found and we conducted a combined surgical-endoscopic approach. Most of the polyps were identified and removed endoscopically (snare polypectomy). Five enterotomies were performed to remove 18 very large polyps (> 3 cm). Finally, a limited portion of the jejunal tract (20 cm) was resected owing to the presence of multiple, large, obstructive polyps. None of the polyps showed cancerous transformation. The shortcomings of the traditional surgical approach include repeated small bowel resections and often early reoperation to manage complications caused by polyps missed at the time of previous surgery. If surgical intervention is required, intraoperative endoscopy is always indicated. Conservative surgical management, the role of intraoperative endoscopy, planned medical follow-up and the need for a national registration system are stressed.