Advanced familial adenomatous polyposis of the duodenum can be difficult to manage. Local treatment performed by endoscopy or surgery typically leads to recurrence, and thus no improvement in disease status or cancer prevention. While virtually all patients with familial polyposis develop duodenal polyps, only 5% will develop carcinoma. It is our approach to perform pancreas-preserving duodenectomy for patients with Spigelman IV disease to prevent carcinoma. This video demonstrates our current technique.