Primary duodenal carcinoma (PDC) is rare and generally considered to have a low resectability rate and dismal prognosis. PDCs as well as other carcinomas in the pancreatic head are treated by curative pancreaticoduodenectomy. In a selected patient, segmental resection or local resection of the duodenum is indicated. Early PDC can be treated by endoscopic resection. There is an increased risk in patients with familial adenomatous polyposis and adenomas in the duodenum.