Cholangiocarcinoma is a primary malignancy of the bile duct and is extremely rare. The location of cholangiocarcinoma (CCA) along the biliary tree is how these tumors are stratified for workup and treatment. Presenting symptoms may range from an incidental finding to painless jaundice to acute cholecystitis depending on the type of CCA. Cross-sectional imaging is key in the diagnosis and staging as biopsy is not always technically feasible. Surgical resection is the mainstay of treatment and can include liver transplantation for perihilar CCA, an extrahepatic bile duct resection, or a pancreaticoduodenectomy. These can be challenging operations with the potential for morbidity. There are data supporting a role for adjuvant chemotherapy and current studies are examining the impact of perioperative treatment.