Both diagnosis and treatment of cholangiocarcinoma are suboptimal. From the former standpoint, fluorescence in situ hybridization, direct cholangioscopy, endocystoscopy, and optical coherence tomography are just a few of the myriad technologies being studied or employed to improve diagnostic yield. From the latter standpoint, most series suggest that fewer than 1 third of patients are resectable for cure, although liver transplantation has increasingly been used in a subset of cholangiocarcinoma patients with extrahepatic disease. Palliation has included chemotherapy which is of dubious value in most series. Instead, a majority of therapies have addressed mechanisms to improve biliary drainage to delay and preclude hepatic failure and minimize the risk of cholangitis. Although the latter has been accomplished with surgery and attempted with external beam irradiation and brachytherapy, percutaneous and/or endoscopic drainage are the most commonly employed methods in widespread use. There are prospective, randomized studies that suggest that bilateral stenting is associated with improved outcomes in bifurcation lesions if plastic stents are placed, that percutaneous transhepatic biliary drainage is more successful than endoscopic stenting in Klatskin tumors, that self-expandable biliary stents have prolonged patency compared with plastic prostheses, and that, if approached with computed tomography and magnetic resonance imaging guidance to preclude contamination of the contralateral undrained system, outcomes are comparable in patients treated with unilateral as opposed to bilateral self-expandable metal stents. There is a single prospective, randomized study suggesting that patients treated with endoscopic or percutaneous stenting in conjunction with photodynamic therapy have prolonged survival compared with stent placement alone.