At present, targeted therapy for cholangiocarcinoma is mostly in the clinical trial stage, such as dasatinib, dapafenib, cetuximab, bevacizumab and so on. Cholangiocarcinoma refers to malignant tumors originated from the epithelium of bile ducts. Based on different anatomical locations, cholangiocarcinoma is classified as intrahepatic cholangiocarcinoma, hepatoportal cholangiocarcinoma and distal cholangiocarcinoma. Because of its lack of prominent clinical manifestations, it is often in the terminal stage when diagnosed. For cholangiocarcinoma patients who cannot undergo surgical resection, chemotherapy is the first choice of treatment, and targeted therapy is also one of the choices. At present, there are a large number of clinical trials of molecular targeted therapy targeting IDH gene, EGFR receptor of FGFR pathway, RAS/RAF/MEK/MAPK pathway and so on. Overseas studies have found that: dasatinib is effective for cholangiocarcinoma with IDH mutation; dapafenib can be used as dual-BRAF and MEK inhibitor to treat patients with BRAF V600E mutation; erlotinib and cetuximab can inhibit EGFR and improve the prognosis of patients with progressed cholangiocarcinoma; bevacizumab in combination with cisplatin, high-dose fluorouracil, and formyltetrahydrofolic acid can alleviate the hepatic metastasis of cholangiocarcinoma. metastasis of cholangiocarcinoma. In conclusion, at present, targeted therapy for cholangiocarcinoma is mostly in the stage of clinical trials. If suspected or diagnosed as cholangiocarcinoma, one should go to regular hospitals for consultation and treatment in time, follow the doctor’s instruction to actively cooperate with the treatment and choose the appropriate treatment plan to avoid delaying the condition. Please strictly follow the doctor’s prescription for medication, and do not use medication on your own.