Abstract: Biliary tract cancer(BTC)is a genetically diverse cancer that includes intra-and extra-hepatic cholangiocarcinomas, as well as gallbladder carcinoma.Most patients with BTC manifest with an unresectable or metastatic disease, and their prognosis is poor.Both surgery and radiotherapy offer limited positive results, and chemotherapy is considered to be the main therapeutic option for these patients.Conventional chemotherapy can reportedly result in a response rate ranging from 10%to 40%.Gemcitabine plus cisplatin combination chemotherapy has been proven to prolong the survival significantly and can become the standard therapy.Although encouraging data on the use of targeted therapies are emerging, further studies are needed to improve the treatment of patients with biliary tract cancer.