Klatskin tumorarises from the common hepatic duct and its bifurcation the bile ducts and is the most common primary malignancy of the biliary tree. The location of the tumor and its close relationship with vascular structures at the hepatic hilum have resulted in a low resectability and high rate morbidity and mortality. Improvementof instrumental diagnostics and operative techniquesallowsto performextendedresectionand complexinterventions onthe liver, bile ductsand vascularstructuresat the hepatic hilum. The role ofchemoradiotherapyandphotodynamic therapyis not fullyunderstood. Thus, questions of treatmentandprognosis of the diseaseare remain relevantandrequire further study.