Previous experience with OLT for hilar CCA has been discouraging, and survival was dismal. This study demonstrates that carefully selected patients with unresectable hilar CCA can achieve long-term survival after OLT. The survival rate obtained with this protocol (5-year actuarial survival of 87%) is comparable with the overall survival rate of liver-transplant recipients at the authors' institution. In comparison, the best survival rate after OLT for hilar CCA reported in the literature is 64.8% at 5 years in a subset of nine patients with negative lymph nodes. In the absence of a control group, it is difficult to assess with certainty the role of a combination of chemotherapy and radiotherapy, but in some patients it seems to prevent or slow progression of the disease while waiting for an available organ. Treatment-related morbidity, although significant, is not prohibitive. Nevertheless, a considerable proportion of treated patients ultimately was found to have advanced disease precluding transplantation. This finding confirms the importance of the staging laparotomy as an essential component of the protocol.