Hepatoportal cholangiocarcinoma is a cancer that occurs in the left hepatic duct, right hepatic duct, left and right hepatic duct bifurcations, and the upper part of the common hepatic duct. the clinical features of these tumors were described in detail by Klaskin in 1965, so they are often called Klaskin tumors. Klaskin’s tumor has been reported to account for approximately 58%-75% of extrahepatic bile duct cancers. Early diagnosis is difficult due to the lack of previous knowledge of the disease and the hidden growth site of the tumor. Patients were already in advanced stage when they came to surgery. Since 1980s, with the development of modern imaging technology, the diagnosis and treatment of hilar cholangiocarcinoma have made great progress clinically. The clinical staging of hilar cholangiocarcinoma is widely cited by the French Bismuth-Corette staging method: 1. Both hepatic ducts are involved. Clinically, the course of cholangiocarcinoma can be divided into 4 stages according to the invasion of hepatic artery and portal vein by the cancer. Stage I: tumor is limited to bile duct without portal vein and hepatic artery invasion; Stage II: tumor involves portal vein and hepatic artery unilaterally; Stage IIIa: tumor involves one side of hepatic artery and bifurcation of portal vein; Stage IIIb: tumor involves one side of portal vein and hepatic innominate artery; Stage IV: tumor involves innominate artery and bifurcation of portal vein. The classic diagnostic pattern of hilar cholangiocarcinoma is: jaundice + dilated intrahepatic bile ducts + normal caliber of extrahepatic bile ducts + empty gallbladder + hilar occupying lesion, which is not difficult to diagnose, but it is mostly in the middle and late stages. B ultrasound is the preferred method to diagnose cholangiocarcinoma of the hilar region, which can show dilated intrahepatic bile ducts, empty extrahepatic bile ducts and gallbladder, and can detect hypoechoic mass shadow of hilar and whether there is cancer embolus in portal vein. For this reason, combined ultrasound and CT should be chosen to accurately determine the location of the tumor and the degree of invasion. Because of the ability to display complete images of the biliary tree inside and outside the liver, hepatobiliary magnetic resonance (MRI) and magnetic resonance pancreaticobiliary imaging (MRCP) are currently the best diagnostic imaging techniques that can clearly show whether grade II bile ducts and common bile duct are involved, and they have replaced and are superior to PTC, ERCP and CT examinations. In the absence of cholangitis, serum CA19-9 values are higher than normal in 86% of patients with hilar cholangiocarcinoma and 71% of patients with CA19-9 values six times higher than normal, so a significant increase in CA19-9 values is helpful for the diagnosis of hilar cholangiocarcinoma. Cholangiocarcinoma-associated antigen (CCRA) is a new antigen found in human cholangiocarcinoma tissues in recent years, and the concentration of serum CCRA increases significantly in cholangiocarcinoma. value. Cytological examination is highly specific but less sensitive when bile is obtained by PTC or ERCP. If bile duct strictures are found to be cancerous by PTC or ERCP, a cytology brush can be placed to repeatedly brush the bile duct strictures to obtain specimens for cytology examination, or biopsy of the lesion can be obtained by pancreaticoduodenoscopy. These two methods have high diagnostic specificity, which can reach almost 100%. Currently, there are many methods to treat cholangiocarcinoma of the hilar region, but surgical resection is the only treatment option for long-term survival. With the development of diagnostic imaging technology, advancement of surgical technique and change of treatment attitude, the surgical resection rate of this disease has improved significantly. Only complete surgical resection of hepatoportal cholangiocarcinoma can provide patients with the only possible chance of cure, and its effect on improving patients’ quality of life is much better than that of various drainage procedures. Therefore, the treatment of hilar cholangiocarcinoma should adopt an aggressive surgical attitude and strive to remove the tumor. Radical resection includes extrahepatic biliary resection, “skeletonization” of blood vessels on the hepatoduodenal ligament, extensive resection of fibrofatty tissue, nerves and lymph on the hepatoduodenal ligament, and if necessary, resection of one liver lobe and reconstruction of hepatic duct-jejunostomy. Most cholangiocarcinomas in the hilar region have caudate lobe infiltration, and caudate lobe resection is necessary for those invading the confluence or left or right hepatic duct. Most scholars believe that prognosis can be improved by aggressive hepatic resection on the basis of correct estimation of the extent of invasion. Palliative treatment includes percutaneous, endoscopic or surgical bypass drainage and endobiliary stent placement, which aims to reduce yellowing and prevent or treat complications such as pruritus, abscesses and liver failure. The methods of placing endobiliary stent tubes are: percutaneous hepatic puncture of the bile duct (PTD) for endobiliary stent placement, transduodenoscopy (ERCP) for endobiliary stent placement, endobiliary stent placement during dissection, and endobiliary stent placement using interventional methods via external drains. In recent years, with the development of interventional techniques, memory alloy stents have been placed in the bile duct through hepatic puncture for drainage, or in the intrahepatic bile duct through the common bile duct via dissection for drainage, with good results. The alloy stent passes through the upper and lower ends of the tumor via the bile duct, so that the obstructed bile flows into the lower part of the hepatic duct and into the duodenum through the stent. However, memory alloy stents are expensive and difficult to perform in general primary hospitals. The advantages of external drainage alone are that it is simple to operate, can drain bile to a certain extent, and play the role of decompression and yellowing; the disadvantage is that the amount of bile loss is large, water and electrolyte disorders are prone to occur, and the nutritional status is deteriorating, which cannot improve the quality of life and prolong the survival of patients. Chemotherapy combined with radiation therapy can improve the local control rate and seems to have certain efficacy in improving the quality of life and prolonging survival, however, there are few cases in most clinical trials and no clear judgment can be made on the efficacy of these adjuvant therapies. Hepatoportal cholangiocarcinoma has the characteristics of intrahepatic metastasis, slow growth and late extrahepatic metastasis, so it has been suggested that it can be a good indication for liver transplantation. The specific approach is to choose in situ liver transplantation, bile duct reconstruction, and parallel Roux-Y anastomosis of the common bile duct with the recipient jejunum to maximize resection of the patient’s proximal bile duct and prevent recurrence. It has been reported abroad that the postoperative survival rate of total hepatectomy with in situ liver transplantation is not significantly different or even better than that of the radical resection group.