Cholangiocarcinoma (CCA) is a primary hepatic neoplasm that arises from malignant transformation of the biliary epithelium. Chronic biliary tree inflammation as occurs in primary sclerosing cholangitis (PSC) is a risk factor for the development of CCA. Surgical resection and liver transplantation following neoadjuvant therapy in patients with early extrahepatic CCA are the only potentially curative modalities. Biliary stenting, chemotherapy, radiation therapy, and photodynamic therapy are palliative treatment options for patients who are not surgical candidates. Liver transplantation following neoadjuvant therapy is an effective therapy for patients with hilar cholangiocarcinoma that is unresectable or arising in the setting of PSC.