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Context Neuroendocrine tumors of the pancreatic ampulla are uncommon. The final diagnosis is based on histology, and at times, it may be difficult to diagnose them pre-operatively since they present with a similar clinical picture to adenocarcinomas of this region. Objective To identify neuroendocrine tumors of the ampulla, as well as their presentation and management. Design A retrospective review of patients treated at a tertiary care institute was performed over a six-year period from 2005 to 2010. Patients Cases with periampullary cancers were investigated. Main outcome measures The case records were scrutinised for the clinical presentation, management and outcomes. Results A total of 4 cases (7.7%) of neuroendocrine tumors of the ampulla were identified from 52 patients with periampullary lesions, at a mean age of presentation of 49 years. The common mode of presentation was progressive jaundice (3 of 4 patients); pancreaticoduodenectomy was performed in 3 patients. One patient underwent palliative endoscopic stenting for metastatic disease. On histopathology, 2 of the patients had poorly differentiated (neuro)endocrine carcinoma (highgrade), and 2 had well differentiated (neuro)endocrine carcinoma (1 low grade and 1 intermediate). All the tumors stained positively with chromogranin A. The patients who underwent pancreaticoduodenectomy are on regular follow-up and remain free of disease. Conclusions Neuroendocrine tumors of the ampulla are distinct entities presenting clinically with jaundice. They stain positive with chromogranin A on histopathology. Pancreaticoduodenectomy should be performed as it is associated with good outcome.