Perihilar cholangiocarcinoma (PHCC) originates from extrahepatic bile duct epithelial cells. It usually occurs at the junction of left and right hepatic ducts and superior hepatic ducts. It is the most common malignant tumor in extrahepatic cholangiocarcinoma. Due to its complex pathogenesis and special anatomical structure, most patients have no obvious special clinical manifestation in the early stage. When symptoms such as jaundice are diagnosed, the disease has progressed to the middle and late stages. Therefore, the diagnosis and treatment of PHCC is a major problem faced by clinicians. For the current treatment of PHCC, surgical resection is still the main effective way. For middle and advanced patients whose tumor cannot be surgically removed, liver transplantation is an effective method. It makes patients have the possibility of long-term survival, and is not limited by tumor stage and liver function. It also can achieve the maximum possible lesion resection. However, the treatment of liver transplantation still faces many controversies and challenges, such as lack of donor liver sources, postoperative complications and tumor recurrence. This article systematically reviews the current status and progress of PHCC liver transplantation.