A 57–year–old man with von Recklinghausen disease was referred to our hospital for melena and severe anemia. A double–balloon assisted enteroscopy revealed a protruding mass with central depression in the third portion of the duodenum. Moreover, contrast–enhanced CT revealed multiple enhancing tumors showing extramural growth, in the duodenum and small intestine. Based on the findings, the duodenal tumor was judged as being the likely source of the bleeding. The patient was then taken for elective partial duodenal resection, which revealed a hard, centrally depressed tumor with extramural growth, measuring 1.5 cm in diameter. In addition, multiple gastrointestinal stromal tumors(GISTs) showing extramural growth were seen throughout the length of the duodenum and small intestine. Histopathologic assessment identified the tumors as GISTs with very low–risk features. This case serves as an important reminder that the possibility of GISTs should be considered in patients with von Recklinghausen disease presenting with gastrointestinal bleeding.