Both hepatic hilum type gallbladder cancer and hilar cholangiocarcinoma are malignant tumors of the biliary tract. In clinic, the two types of cancer are easy to be misdiagnosed. They have high degree of malignancy, limited treatment modalities, low surgical R0 resection rate, and poor prognosis. When gallbladder cancer invades the hepatic hilum area, it and extrahepatic cholangiocarcinoma all are perihepatic tumors. It often causes similar symptoms such as jaundice with liver damage. However, they are difficult to be diagnosed in early stage from the clinical symptoms. Whether or not to drain preoperatively is a common point of controversy in biliary surgical diseases. It is also controversial points such as the advantages and difficulties of minimally invasive access and how to solve them as well as the extent of surgical resection. All of these will be discussed in detail in this paper. The article will identify the two types of diseases, and at the same time, put forward some reflections and outlooks in order to provide clinical references.