Patients with duodenal polyps associated with familial adenomatous polyposis (FAP) have a considerable risk of developing duodenal carcinoma. Prophylactic resection of the duodenum for Spigelman stage III disease is the treatment of choice to prevent progression to cancer. Pancreaticoduodenectomy and pancreas-preserving total duodenectomy (PPTD) are the techniques that have been described for the surgical treatment of duodenal polyposis. We report the first case of laparoscopic PPTD in a patient with previous total colectomy for FAP and Spigelman stage III duodenal polyposis. A laparoscopic total dissection of the duodenum was carried out and the restoration was achieved performing pancreatico-biliary-jejunostomy and gastrojejunostomy. The postoperative period was uneventful. Laparoscopic PPTD can be performed safely in selected cases for the management of FAP.