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To review our experience in patients undergoing operative treatment for duodenal polypoisis associated with familial adenomatous polyposis with an emphasis on operative approach and long-term outcomes.Duodenal polypoisis associated with familial adenomatous polyposis patients undergoing operative treatment were studied retrospectively excluding patients with preoperative duodenal cancer.Of 767 patients in the database, 63 (8.2%) patients underwent operative treatment: 42 (67%) pancreas-sparing duodenectomy, 15 (24%) pancreatoduodenectomy, and 6 (9.5%) segmental duodenal resection; the majority for Spigelman stages III and IV polyposis. Overall 9.6% had adenocarcinoma postoperatively (28.6% in the pancreatoduodenectomy group; P = .01). The proportion of Spigelman stages III and IV with cancer were 9.5% and 6.5%, respectively. Pathologic upgrade to cancer in patients with low grade dysplasia and high-grade dysplasia on preoperative biopsy was 5.7% and 6.7%, respectively (P = .13). At a median follow-up of 16 years, 7.7% needed a second duodenal polypoisis associated with familial adenomatous polyposis-related operation. Progression to high grade dysplasia or cancer in the stomach occurred in 15.4% of patients. Median overall survival and recurrence-free survival was at least 16 years and 15.6 years. No significant group-based differences were noted on follow-up.The majority of patients with duodenal polypoisis associated with familial adenomatous polyposis can achieve long-term, cancer-free survival with organ-preserving approaches (pancreas-sparing-duodenectomy and segmental-duodenal-resection) with survival not dependent on the type of resection.