Type 1 Neurofibromatosis (von Reckinghausen’s disease) has been associated with several gastrointestinal tumor complications, either benign or malignant. We report a case of a middle age man who had Type 1 Neurofibromatosis and presented with acute intestinal obstruction. Laparotomy revealed multiple nodular lesions along the small intestine with two larger nodules which were resected. Histopathological examination of the nodules confirmed it as gastrointestinal stromal tumors (GIST) with malignant potential. The association between neurofibromatosis and GIST is discussed.