A case report is given of a 24 year old patient who had relapsing massive gastrointestinal bleeding from varices in the duodenum and jejunum, due to occlusion of the mesenterial vein. There is only one similar case described in the literature. 97 patients with varices of the small intestine have been described; in 41 of these cases there was extrahepatic, in 47 intrahepatic portal hypertension. In 60 of the 91 cases varices were found in the duodenum, less frequently in the ileum or jejunum too. In another 10 cases varices had formed around an ileostomy. In 1/3 of all cases abdominal surgery had preceded formation of varices in the small intestine. Pathogenesis, diagnosis and therapy are discussed.