Cholangiocarcinoma accounts for 2% of all malignancies in the United States and 40-60% are considered hilar arising at the hepatic duct bifurcation. The prevalence of cholangiocarcinoma, however in patients with a diagnosis of primary sclerosing cholangitis has been reported to be present in 7 to 13% and develops at a rate of 1% per year. The diagnosis of cholangiocarcinoma can be made by interpretation of brush cytology or biopsy of a mass identified by computed tomography or magnetic resonance imaging. Non-surgical therapy has been associated with very poor survival long-term, 1 and 5 year survival of 26% and 4%, respectively. Surgical excision significantly improves survival. An R0 resection, that is a resection where no known tumor is left behind, increases survival to 60% at 2 years. Resection is not possible, however in some patients due to extension of disease to both hepatic lobes or risk from liver resection due to underlying cirrhosis. Liver transplantation has been proposed in these circumstances. In the United States 6 to 7% of liver transplants are performed for malignancy. The majority of these are for the presence of hepatocellular carcinoma and only a small minority are for cholangiocarcinoma. For the period of 1988 to June 2006, UNOS reports that 213 patients were transplanted for the diagnosis of cholangiocarcinoma compared to 3,344 patients transplanted for hepatocellular carcinoma. Since the introduction of MELD, a provision for an exception for patients with hepatocellular carcinoma has been established to increase the assigned MELD score to increase the ability of patients with a curable malignancy to achieve transplantation prior to spread of the disease. The criteria used to establish these criteria were developed by Mazzafero and colleagues and are referred to as the Milan criteria based on a 1996 publication detailing the outcomes in 48 patients that underwent transplantation that met these criteria. Several reports historically have shown poor outcomes after liver transplantation for cholangiocarcinoma, far below the outcome for liver transplantation for other diagnoses. The reasons for patient death in these earlier reports were due to a high risk for recurrence. The stage of disease in these cases were advanced (the majority with stage III or IV tumors). Two centers have subsequently demonstrated that with selection of patients with only earlier stage disease and the addition of neoadjuvant therapy, the survival after liver transplantation for cholangiocarcinoma approaches the outcomes for other diseases. It appears reasonable in the current setting to reconsider application of liver transplantation to patients with cholangiocarcinoma. Currently there are recommendations from the Liver/Intestine committee of UNOS out for public comment that suggest an increase in MELD score for patients that meet criteria similar to the Mayo and Nebraska protocols. In summary, although in the past liver transplantation for cholangiocarcinoma was associated with high rates of malignancy recurrence, most of these patients in earlier reports had in fact advanced disease at the time of transplantation. Better selection of patients for patients with stage I or II and the introduction of neoadjuvant therapy by 2 programs has resulted in acceptable outcomes in the same range as those patients in whom resection is possible.