Hepatoportal cholangiocarcinoma refers to malignant tumors occurring in the common hepatic duct, left and right hepatic ducts and their confluence, also called proximal cholangiocarcinoma or high-grade cholangiocarcinoma, which accounts for about 60% of all cholangiocarcinomas. The cancer tissue can easily invade the hepatic artery and portal vein and form cancerous thrombus, which can lead to liver metastasis and lymph node metastasis, and also invade to the adjacent organs of pancreas and gallbladder. Jaundice is the earliest manifestation of hepatoportal cholangiocarcinoma, and the jaundice deepens progressively. Extrahepatic cholangiocarcinoma is often misdiagnosed as “infectious hepatitis” and treated, and even patients are already in advanced stage when they go to surgery.