The incidence of cholangiocarcinoma is on the rise. This may be related in part to higher detection rates secondary to sophisticated endoscopic modalities. These tumors pose a significant diagnostic dilemma. High index of suspicion, careful interpretation of serum markers and utilization of endoscopic techniques, including endoscopic retrograde cholangiopancreatography and endoscopic ultrasound, ... [Show full abstract] help to establish the diagnosis. Imaging modalities are crucial in the evaluation of these tumors. They help to define the extent of the native lesion, which may dictate its resectability, as well as evaluate for metastasis. Therapeutic options are somewhat limited, short of surgical resection. Newer chemotherapeutic agents, as well as endoscopy-targeted therapy, may improve the overall treatment success rate, although experience is somewhat limited at this time. Endoscopic intervention is essential for palliation.