Duodenal varices are an uncommon but serious manifestation of portal hypertension. Our management of three patients with massive bleeding due to duodenal varices stimulated a review of this subject. Thirteen cases of this condition were previously reported. Endoscopic examination of the entire duodenal mucosa is essential to document bleeding from duodenal varices. Medical therapies, including vasopressin and endoscopic sclerotherapy, have had limited success in controlling active duodenal variceal bleeding. Duodenal varix suture ligation or resection also resulted in a high rate of rebleeding. End-to-side portocaval shunt was the most effective procedure in stopping acute and subsequent bleeding in patients with duodenal varices. Despite therapy with or without portosystemic shunt, mortality risk is high in Child's class C patients and in patients with emergency duodenal variceal bleeding.