The occurrence of both gastrointestinal carcinoid and von Recklinghausen's disease in the same patient is uncommon. There seems to be a predilection for ampullary carcinoids in these patients. This article reports the sixth case, including a review of the literature and a brief discussion of the implications of this finding. A carcinoid tumor should be suspected in any patient with von Recklinghausen's disease presenting with gastrointestinal bleeding, obstruction, abdominal pain, and particularly obstructive jaundice.