Cholangiocarcinoma refers to malignant tumors occurring in the lining epithelium of the bile duct system. Intrahepatic cholangiocarcinoma originates from the lining epithelium of any part of the intrahepatic bile duct and its branches to the interlobular biliary tree; extrahepatic cholangiocarcinoma is divided into hilar cholangiocarcinoma and distal cholangiocarcinoma based on the confluence of the cystic duct and common hepatic duct. The causes of cholangiocarcinoma are still unclear. Risk factors for its development have been reported in the literature, including advanced age, bile duct stones, bile duct adenoma and bile duct papillomatosis, Caroli disease, common bile duct cyst, viral hepatitis, cirrhosis, primary sclerosing cholangitis, ulcerative colitis, chemical toxins, smoking, and Schistosoma haematobium or Schistosoma chinense infection. Clinical manifestations of bile duct cancer vary depending on the location and size of the tumor. Patients with intrahepatic cholangiocarcinoma often have no special clinical symptoms in early stage, but with the progress of the disease, abdominal discomfort, abdominal pain, weakness, nausea, epigastric mass, jaundice, fever, etc. Jaundice is rare. Patients with hilar or extrahepatic cholangiocarcinoma can mostly develop jaundice, which gradually deepens with time, light and grayish stools, dark yellow urine and itchy skin, often accompanied by general manifestations such as lethargy, fatigue and loss of body mass. Pain in the right upper abdomen, chills and fever suggest the presence of cholangitis. In the case of biliary obstruction, liver function tests suggest elevated bilirubin, alkaline phosphatase and γ-glutamyl transpeptidase. Aminotransferases may be elevated and may be significantly elevated in the presence of cholangitis. Long-term biliary obstruction can lead to a decrease in fat-soluble vitamins (A, D, E and K) and a prolongation of prothrombin time. As the disease progresses, albumin, hemoglobin and lactate dehydrogenase levels may decrease. Surgical resection is the primary treatment for cholangiocarcinoma. As long as cholangiocarcinoma can be resected radically, the patient’s general condition can tolerate it and there is no distant metastasis, surgical treatment should be actively carried out to obtain radical resection. For those who cannot be resected, neoadjuvant chemotherapy may lower the stage of tumor and increase the chance of radical surgical resection. The outcome of surgery mainly depends on the location of the tumor and the extent of tumor infiltration into the bile duct, the tumor-free margin of surgery and the presence of lymphatic metastasis. The main reasons why long-term survival rates for surgical treatment remain suboptimal include the fact that approximately 5% of bile duct cancers are multifocal, 50% of patients have associated lymph node metastases, and 10% to 20% of patients have peritoneal and distant metastases. In the past, it was believed that liver transplantation did not improve the survival rate of patients with cholangiocarcinoma. Recent studies have shown that preoperative liver transplantation with radiotherapy can significantly improve the long-term survival rate of patients after transplantation. Neoadjuvant radiotherapy can achieve a 5-year tumor-free survival rate of 65% after liver transplantation for patients with cholangiocarcinoma. However, the long-term survival rate was significantly lower in patients with tumor diameter >3 cm, distant metastases, transperitoneal tumor puncture biopsy and previous history of malignancy.