Somatostatinoma of the gastrointestinal tract is a rare finding, especially arising from the ampulla of Vater. We present a man recently diagnosed with rectal adenocarcinoma who was found on imaging to have an ampullary mass. Histology and immunohistochemical staining of the biopsied tissue were consistent with a nonfunctioning somatostatinoma. The patient had neurofibromatosis, which have been reported in patients with ampullary somatostatinomas. Our case highlights the importance of gastrointestinal findings in those with underlying genetic conditions.