Duodenal endocrine tumors are rare and represent 2% of all gastrointestinal endocrine neoplasms. Carcinoids of the ampulla of Vater are the most rare primary ampullary tumors. There was noted a frequent association of the endocrine tumors with type 1 neurofibromatosis also known as von Recklinghausen disease. To the best of our knowledge there are only 8 cases of papilla duodenalis minor carcinoids described in the literature. Authors describe herein the first carcinoid of papilla duodenalis minor case associated with multiple synchronic jejunal leiomyomas and von Recklinghausen disease, manifested with proximal intestinal obstruction and resolved by pancreatoduodenectomy (Kausch-Whipple procedure).