Biliary tract cancer (BTC) are highly aggressive and fatal malignancies. As one of the leading cause of death in Republic of Korea, BTC amoung various malignancies has been one of major public health concerns in the country. Although BTCs, including intrahepatic, perihilar, and distal cholangiocarcinoma, are relatively low-incidence malignancies compared to other cancer, but they represent a major health problem in endemic areas like Korea and Asia contries; moreover, the incidence of intrahepatic cholangiocarcinoma is rising globally. Surgery is the only curative treatment. The optimal surgical approach depends on the anatomical site of the primary tumour, and the best outcomes are achieved through management by specialist multidisciplinary team. Unfortunately, most patients present with locally advanced or metastatic disease. Only up to 20% of patients are diagnosed in early-stage, suitable for the curative surgery. Despite the surgery performed with potentially-curative intent, recurrence rates are high, around 60-70% of patients expected to have disease recurrence. In this review, we present an update of the causes, diagnosis, and treatment with a focus on chemotherapy of BTC.