The term cholangiocarcinoma (CCA) includes a group of malignant tumors that develop in the efferent bile ducts and are characterized by a high degree of heterogeneity. These differences between intrahepatic, perihilar and distal CCAs run through all aspects of the disease including the etiology, pathogenesis, symptoms, diagnostics and treatment. This review article presents the current developments in this field of diseases. We highlight surgical innovations in the clinical routine and the application of new systemic forms of treatment to augment the oncological radicality of surgery.