BACKGROUND: Pancreas-preserving total duodenectomy (PPTD) was introduced as a replacement for pancreatoduodenectomy (PD) for familial adenomatous polyposis (FAP). This study analysed the results of PPTD in the Netherlands and reviewed the relevant literature. METHODS: All 26 patients who underwent PPTD for FAP in four centres in the Netherlands between January 2000 and January 2007 were compared with a group of 77 patients who had PD for ampulla of Vater adenocarcinoma at one centre during the same interval. RESULTS: Morbidity rates were similar after PPTD for FAP (16 patients, 62 per cent) and PD for ampulla of Vater adenocarcinoma (44 patients, 57 per cent) (P = 0.694). One patient (4 per cent) died after PPTD and two (3 per cent) after PD. A review of the literature, including patients from the present study, found that 71 patients had PPTD, with postoperative morbidity in 36 (51 per cent) and one death (1 per cent). In publications containing a total of 94 patients who underwent PD for FAP, 43 (46 per cent) developed complications and three (3 per cent) died. CONCLUSION: PPTD has similar short-term results to PD in terms of morbidity and mortality.