BACKGROUNDNeuroendocrine tumours very rarely occur in the biliary tract; information about them is limited.AIMSTo present the clinical characteristics and prognosis of curatively resected biliary neuroendocrine tumours.METHODSReview of medical records dated between 2000 and 2010 of 20 patients from three medical centres with biliary neuroendocrine tumour based on curative resection.RESULTSBased on the World Health Organization 2010 classification, five and one patients had neuroendocrine tumour grades 1 and 2, seven had neuroendocrine carcinoma, and seven were diagnosed with mixed adenoneuroendocrine carcinoma. The locations were the following: seven in the gallbladder, four in the extrahepatic bile duct, and nine in the ampulla of Vater. Lymph node and hepatic metastases were noted in 11 and 4 patients, respectively. Fourteen patients experienced recurrence; most had recurrence in the liver. Patients with neuroendocrine tumour grade 1 had a lower rate of recurrence compared to others (p=0.001). The median disease-free and overall survival times were 5.8 (0.4-53.6) and 13.7 (1.9-102.1) months for all four subtypes. However, the median disease free and overall survival rates of neuroendocrine tumours were significantly longer than those of neuroendocrine carcinomas or mixed adenoneuroendocrine carcinoma.CONCLUSIONSPatients with biliary neuroendocrine tumour showed extremely different clinical outcomes according to histopathologic subtypes by World Health Organization 2010 classification.