We analyzed the features of 7 patients who underwent orthotopic liver transplantation (OLT) from April 2001 to April 2006 for incurable hepatic alveolar echinococcosis (AE) in view of the technical features of the OLT, incidence, and type of complications, as well as patient survival. All 7 patients had biliary diseases that resulted from parasitic involvement of the hilum or a large parasitic lesion that invaded the lobes of the liver due to parasitic involvement of the hepatic veins, which are all typical syndromes of hepatic AE. Five patients had previously undergone surgery for hepatic AE and were at the end stage of liver functional disorder. Four patients' operations were performed under venovenous bypass. The mean duration of surgery was 9.1 hours. After transplantation, one patient experienced biliary leakage but was successfully treated with reoperation. One patient died 21 days later of septicemia caused by pneumonia and multiple organ failure, and another patient died 3 months after OLT from heart failure. The other 5 patients are alive and in good condition after transplantation. Although the transplantation procedure was more difficult than usual in these patients, most achieved prolonged survival and a good quality of life. This study suggests that OLT is feasible for incurable AE of the liver and that this procedure ensures a good clinical outcome.