Liver transplantation for treating cholangiocarcinoma (CCA) has potential benefits in terms of accomplishing radical resection even in patients with unresectable tumor and treating underlying disease such as primary sclerosing cholangitis (PSC). Despite this ideal rationale, the initial results of liver transplantation alone for CCA have been uniformly poor. Because of a high recurrence rate and a poor survival rate, CCA has been viewed as an absolute contraindication for transplantation. However, from the 1990s, liver transplantation following high dose neoadjuvant radiotherapy with chemotherapy has achieved excellent results, in a few medical centers, for selected patients with unresectable hilar CCA arising in the setting of PSC. Especially, the Mayo Clinic reported a 5-year survival rate of 73% for 120 patients in 2010 by performing neoadjuvant therapy and liver transplantation. We reviewed the results of liver transplantation in patients with hilar CCA and the protocol of the Mayo Clinic to consider the feasibility of liver transplantation for Korean patients with hilar CCA. Although liver transplantation with neoadjuvant therapy cannot be applied to most Korean patients due to the different disease entities and the good results with performing radical surgical resection, it can be considered as an alternative curative treatment option for selected patients with anatomically unresectable hilar CCA or CCA arising in the setting of PSC.