Surgical resection or liver transplantation (LTx) are the only available treatments that offer a potential for long-term survival or cure in cases of hilar cholangiocarcinoma. Hilar resection in combination with partial hepatectomy and caudate lobectomy is regarded as the current treatment of choice. Overall 5-year survival rates range from 9% to 28%, and reach as high as 24–43% in R0 resections. Five-year survival rates in the very limited experience with LTx in hilar cholangiocarcinoma are not dramatically worse than those after resection. However, hilar cholangiocarcinoma is not at present an accepted indication for LTx given both the good results of LTx for benign diseases and the dramatic organ shortage. When compared with the prognosis of other gastrointestinal tumours, these survival rates are encouraging in the setting of an otherwise unresectable malignancy. As such, and considering the fact that it may represent the only possibility for cure, the general exclusion of patients with cholangiocarcinomas as candidates for LTx does not seem to be justified. Furthermore, recent advances in multimodal tumour therapy seem to be most promising in combination with LTx. Prospective studies are required to elucidate the influence of better patient selection and the role of multimodal treatments on the outcome of LTx in hilar cholangiocarcinoma. If the encouraging data achieved with neoadjuvant therapy prior to LTx are confirmed by further studies, we foresee that renewed interest in LTx for hilar cholangiocarcinoma could arise.