Objective: To investigate the clinical features and prognostic factors of extrahepatic biliary neuroendocrine neoplasms(EB-NENs). Methods: The clinical data and survival of 21 patients with EB-NENs admitted from May 2014 to May 2018 were analyzed retrospectively. Results: These 21 EB-NENs patients accounted for 1.6% (21/1313) of all biliary tract neoplasms treated during the study period. Seven (33.3%) cases had lymph node metastasis. Five (23.8%) suffered from distant metastasis. The follow up time was (4~46.5) months with median survival time of (23.23 ±4.17) months. Univariate analysis showed that the risk factors for survival were tumor TNM stage (χ²=9.066, P=0.003), lymph node metastasis(χ²=6.399, P=0.011) and distant metastasis (χ²=9.808, P=0.002). By multivariate analysis, independent risk factors were tumor TNM stage(P=0.008, RR=3.003, 95% CI: 1.332~6.774), lymph node metastasis(P=0.023, RR=5.382, 95% CI: 1.261~22.971) and distant metastasis or not(P=0.007, RR=7.423, 95% CI: 1.730~31.851). Conclusion EB-NENs was a rare malignant tumor with poor prognosis. Tumor TNM stage, lymph node metastasis and distant metastasis were independent prognostic factors for EB-NENs patients.