We report a 51-year-old woman with known neurofibromatosis of von Recklinghausen, who presented twice with melena, caused by a jejunal leiomyoma. The diagnosis was made by arteriography and confirmed by surgery. In order to exclude an intestinal smooth muscle tumour we believe that mesenteric angiography should be the initial procedure of choice in patients with neurofibromatosis presenting with a gastrointestinal bleeding. Genetic analysis suggests that the association of neurofibromatosis and intestinal leiomyoma is more than fortuitous.

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