Ampullary Neuroendocrine tumor (ANET) is a rare GI malignancy, representing less than 1% of GI neuroendocrine tumors and less than 2% of ampullary tumors. Traditional treatment is often a pancreaticoduodenectomy; however, local and endoscopic resections have been successful. We report a rare case of ANET in a 21-year-old Burmese man who presented with a 6-year history of non-specific intermittent abdominal pain who was successfully managed through transduodenal ampullectomy. At 24 months postoperatively he remains disease and symptom free. ANET is a rare cause of recurrent abdominal pain, and local excision of small ANETs can be an alternative, less morbid treatment for young patients. We follow the case with a brief review of the literature.