Cholangiocarcinoma is the second most common hepatobiliary tumour. Even if it is a rare tumour, its incidence is increasing over these last decades, probably due in part to a better knowledge of the disease and to an improvement of the diagnosis. Accurate diagnosis and staging are key steps to determine the appropriate treatment. The only curative treatment of this cancer is surgical resection. To date, no neoadjuvant or adjuvant treatments have ever proved any survival benefit, and are not recommended outside clinical trials. Liver transplantation (with or without neoadjuvant treatment) can be an option for highly selected cases. Unfortunately, these tumours are generally diagnosed at an advanced stage or are unresectable. For most of these patients, palliative therapeutic options exist and are in further development, based on multimodal promising combinations including chemotherapy, targeted agents, radiation, endoscopic stenting and photodynamic treatment.