The incidence of extrahepatic cholangiocarcinoma is increasing worldwide and is often in an advanced stage at diagnosis and difficult to treat. The TNM (tumor node metastasis) cancer staging system predicts survival on the basis of tumor histopathology and the presence of distant metastases. However, numerous prognostic factors have been described that are not included in the TNM system. This review focuses on the prognostic significance of clinical, surgical, and histopathological factors as reported in the literature. Overall, the most important independent prognostic factors for long-term survival are negative surgical margins, lymph node status, and differentiation grade of the tumor. Further improvement of staging systems and identification of prognostic factors are crucial if we are to better select patients for surgical and adjuvant therapy and, hence, increase the rate of curative resections.