Hilar cholangiocarcinoma was accepted as an indication for liver transplantation at the beginning of the transplantation era. Owing to disappointing long-term results for this indication, and in parallel, encouraging results in patients with benign disease, hilar cholangiocarcinoma has generally not been accepted as an indication for liver transplantation in recent years. To improve results, more aggressive approaches have been used: "abdominal organ cluster transplantation" and "extended bile duct resection", which lead to increased long-term survival rates. However, with improving results after conventional extrahepatic bile duct resection in combination with partial hepatectomy, extended procedures in combination with liver transplantation never became a real option in the treatment of hilar cholangiocarcinoma. However, new awareness of liver transplantation in the treatment of this cancer has been raised for patients with hilar cholangiocarcinoma in the context of underlying liver diseases such as primary sclerosing cholangitis, which preclude liver resection. Current results show increased survival figures, in particular in well-selected patients with early tumor stages. Further improvements in long-term survival may be reached with new adjuvant and neoadjuvant protocols. Patients with neoadjuvant radiochemotherapy show long-term results similar to those for liver transplantation for other indications. Also, photodynamic therapy and the use of new antiproliferative immunosuppressive agents may be an approach for further improvement of the long-term results. Currently, liver transplantation for the treatment of hilar cholangiocarcinoma should be restricted to centers with experience in the treatment of this cancer and should be taken into consideration in patients with contraindications to liver resection.