Background : Neuroendocrine tumor of the ampulla of Vater is extremely uncommon and is generally a low-grade endocrine cell tu¬mor. The merits of radical vs. local resection remain uncertain.

Methods : A 46-year-old female patient presented with dyspepsia lasting for 3 months. Papilla of Vater that was tumor-like macroscopically was seen in the second part of the duodenum in endoscopic retrograde cholangiography. Biopsy was histologically diagnosed nonspecific fibrosis. After biopsy, she developed complicated bleeding from the Ampulla of Vater biopsy site. Patient condition was stabilized with repeated endoscopic bleeding control. No lymphadenopathy or visceral metastasis was found on an abdominal CT scan and MRI.

Results : Transduodenal Ampullectomy was done. Ampullary tumor was diagnosed as neuroendocrine tumor, low grade. All margins of resection were negative.

Conclusions : Transduodenal ampullectomy may be the alternative step instead of endoscopic snare papillectomy in the management of neuroendocrine tumors of the ampulla of Vater in selected patients such as those with a well differentiated, low-grade, small tumor without regional/distant metastasis.