Duodenal varices (DV) are rare. We present a review of published cases with emphasis on the management and outcome, as illustrated by our own cases, which reflects the experience reported in the literature. The diagnosis of DV must be considered in patients with gastrointestinal bleeding. Two-thirds of all reported cases have portal venous hypertension caused by hepatic cirrhosis. In the remaining one-third prehepatic portal hypertension as a consequence of either a compromised portal venous circulation (caused by perivenous tumor or inflammation) or a primary haematological disease is the underlying cause. Previously, duodenoscopy has often failed to detect and correctly interpret DV, and was similarly unsuccessful in our case. This case report demonstrates the problems and shortcomings in the management of DV and documents a hither to unreported cause. Treatment depends on the severity of bleeding. When conservative measures cannot control the haemorrhage, emergency laparotomy may be indicated. The type of surgery should be chosen according to the aetiology, site and extent of the bleeding DV. Among 112 reported cases of DV, information on outcome exists for only 35 patients who presented with haemorrhage. The aetiology was liver cirrhosis in 26 of these patients, 10 of whom had a fatal outcome, and prehepatic portal hypertension in the remaining 9, 1 of whom had a fatal outcome.