This article provides an overview of recent advances in the diagnosis and treatment of extrahepatic cholangiocarcinomas (EH-CCAs), focusing on the role of endoscopy, surgery, and transplantation. It reviews optimal evaluation and management of patients with EH-CCA, including a careful integration of clinical information, imaging studies, cytology and/or histology, as well as a coordinated multidisciplinary approach. It reviews additional therapy such as radio-or chemotherapy either in the neoadjuvant or adjuvant setting. Furthermore, it addresses palliative approaches as well as emerging targeted therapy and immunotherapy. EH-CCAs account for nearly 90% of biliary tract malignancies and present an ongoing challenge for hepatobiliary surgeons.