Abstract: Biliary tract carcinoma (BTC) is a common malignancy in human digestive system. The lethality of BTC is essentially attributed to the asymptomatic growth of BTC and the lack of effective diagnostic options. The disease is therefore usually detected at an advanced stage and lost the opportunity of surgical therapy. Conventional chemotherapy and radiation therapy have been notably ineffective against BTC. There is now a real and urgent need to focus on developing novel and potent therapeutic strategies against BTC. Human epidermal growth factor receptor 2/neu (HER2/neu) is a member of the ErbB receptor tyrosine kinase family. HER2/neu is associated with tumor proliferation, invasion, and metastasis that would impact in a significant way on clinical outcome. HER/neu may become a new therapy target of BTC.