Cholangiocarcinomas (CCC) are rare tumors with an incidence of 2-4/100,000 per year. They are a heterogeneous group of neoplasias that include the most common perihilar or Klatskin tumor (60%), the intrahepatic (peripheral) CCC, the extrahepatic bile duct cancer, the gallbladder cancer and the cancer of the ampulla of Vater. At the time of diagnosis only 20% of patients can be treated by surgery, that offers the only chance for cure. Due to high recurrence rates liver transplantation is not indicated. Patients with advanced unresectable carcinoma have a dismal prognosis with an overall survival rate of only 6-8 months. Neither chemotherapy nor radiation therapy improves survival. In patients not eligible for curative surgery prevention or treatment of cholestatis is the main objective. This can be achieved endoscopically, percutaneously or by surgical biliodigestive anastomosis. Palliative chemotherapy results in response rates up to 20%. The most frequently used agents are 5-FU and Gemcitabine that can be combined with external or internal radiation. By combining different treatment modalities significant survival can be achieved in some patients. Evidence Based Medicine studies are needed before treatment strategies can be recommended for clinical practice.