Neuroendocrine tumors (NETs) or carcinoids represent a small percentage of gastrointestinal neoplasms (2%). The ileum (41.8%), rectum (27.4%) and stomach (8.7%) are the most common locations. We present an unusual case of NET due to its origin in the ampulla of Vater, as an extremely rare cause of biliary obstruction. A bibliographic review of the current recommendations of management and treatment for this case, different from other locations of the gastrointestinal tract is carried out. The curative treatment of choice for early-stage Vater ampulla NETs is Whipple surgery with lymphadenectomy due to Its high rate of lymphatic and metastatic dissemination, regardless of size and histological grade, although there are no updated official guidelines.