Neurofibromatosis type-1 is a rare autosomal dominant disorder, caused by a mutation in the tumor suppressor gene NF1 which leads to a decreased production of the protein neurofibromin. We present a 59-year-old female with a history of von Recklinghausen’s disease under study for symptoms of toxic syndrome accompanied by anemia and melena. The abdominal Computed Tomography (CT) scan revealed confirmed a tumor of the left adrenal gland, 6 cm in diameter and images compatible with GIST in the small intestine affecting several intestinal loops. The study is completed with the performance of colonoscopy and PET/CT. After preoperative preparation with œ- and ß-adrenoreceptor blockade, both adrenal tumor and ileoterminal GIST were resected. There were no post-surgical complications and the patient was released 10 days after the procedure.