Cholangiocarcinoma is a rare but aggressive malignancy of the bile ducts. While these tumors can occur anywhere along the biliary tree, extrahepatic cholangiocarcinomas (ECC) predominate and are further anatomically subdivided into perihilar or distal ECCs. This disease typically presents with biliary obstruction, resulting in jaundice or pruritus. Surgical resection of ECC represents the only treatment modality to offer potential cure and long-term survival, though unfortunately the majority of patients are not candidates for surgery at the time of diagnosis. Consequently, optimal management requires comprehensive multidisciplinary evaluation and intervention. In this chapter, we review the pathophysiology, risk factors, diagnostic challenges, and therapeutic strategies employed in the management of ECC.