Jejunal varices were a cause of recurrent gastrointestinal hemorrhage in an 18-year-old man with common variable immunodeficiency and nodular lymphoid hyperplasia of the small intestine. Despite numerous procedures, including upper gastrointestinal endoscopy, colonoscopy, arteriography, and exploratory laparotomy no active bleeding site was identified until superior mesenteric angiography demonstrated thrombosis of the superior mesenteric vein with an extensive collateral circulation through mesenteric varices. At laparotomy, mesenteric lymph nodes up to 4 cm in diameter seemed to be compressing the superior mesenteric vein. Histological examination of a node revealed reactive hyperplasia with prominent germinal centers. After resection of varices in a 20-cm length of proximal jejunum, there has been delayed, but complete resolution of bleeding in a 17-month follow-up.