A combined surgical and endoscopic approach for managing extensive polyposis in a child with Peutz-Jeghers syndrome (PJS) is presented. Application of this technique offers the following advantages over conventional surgical practice -- 1) an accurate assessment can be made of the extent of intestinal polyposis; 2) small polyps can be easily removed endoscopically; 3) endoscopy can direct the operating surgeon to selected enterotomy sites for open excision of larger polyps and 4) multiple laparotomy, extensive intestinal resection(s) and the potential threat of short-gut syndrome in PJS patients may be avoided.