Cholangiocarcinoma is a primary liver tumor arising from the small bile ducts within the liver. According to its different location, clinical features, frequency of metastases, treatment modalities and prognosis, intrahepatic cholangiocarcinoma should well be differentiated from proximal bile duct carcinoma. To date, there is no therapeutic measure with curative potential apart from surgical treatment. Partial hepatectomy is the treatment of choice. It is of overriding importance to achieve microscopically tumor-free margins. However, only few patients treated in an early stage have a prolonged recurrence-free survival or a chance for cure. Liver transplantation is not an alternative therapeutic option for unresectable cholangiocarcinoma, due to early tumor recurrence in almost all recipients. Liver transplantation has a place in preventing cholangiocarcinoma in primary sclerosing cholangitis, although the timing of replacement is still a matter of debate. Results of surgery need further improvement by adjuvant or neoadjuvant treatment protocols.