Duodenal adenocarcinoma remains the leading cause of cancer death in familial adenomatous polyposis patients following colectomy. Stratification based on Spigelman’s criteria provides a means for determining therapy. Spigelman stage IV patients have been selected for pancreas-sparing duodenectomy. Twentyone patients underwent resection between 1992 and 2004, with a mean age of 58 ±11 years. The mean time from colectomy to duodenectomy was 27 ±13 years. Invasive cancer was found in the distal duodenum in one patient. Operative time averaged 327 ±61 minutes with a mean blood loss of 503 ± 266 ml. There was no mortality, and eight patients (38%) had 14 complications: six (29%) with delayed gastric emptying, four (19%) with biliary/pancreatic anastomotic leak, one with pancreatitis, and one with wound infection. There were two reoperations: one for delayed gastric emptying and one for an early biliary leak. Mean length of stay was 15 ±10 days. Two late complications occurred: a stomal ulcer and an intestinal obstruction at 48 and 24 months, respectively. Mean follow-up was 79 months (range, 3–152 months). Two patients developed polyps in the advanced jejunal limb and were endoscopically treated. Pancreassparing duodenectomy represents a definitive treatment for advanced duodenal polyposis and can obviate the need for pancreaticoduodenectomy.