Objective: To explore the indications and clinical value of orthotropic liver transplantation(OLT)in patients with advanced hepatic alveolar echinococcosis(HAE). Methods: The clinical data of 12 patients with advanced HAE who received OLT from January 2001 to December 2017 were retrospectively analyzed. Among them, there were 7 males and 5 females, the age ranged from 16~58 years(median 42 years). The preoperative hepatic functions of 12 patients were 2 Child-Pugh class A, 3 class B and 7 class C, and the median model for end-stage liver disease(MELD)score was 19.5(8~23). Results: The mean time of OLT procedure and anhepatic phase in 12 patients were 456.25(456.25±44.98)min and 79.17(79.17±10.01)min respectively. The median intraoperative blood loss and hospital stay times were 1000 ml(600~4000 ml), and 29.5(15±58)days respectively. All the patients were followed-up for 0 months to 207 months. Among the 12 patients who underwent OLT, 2 recipients died of incurable infection of pulmonary infection on day 23 post-OLT, and multiple organ failure on day 32 post-OLT, respectively. One case developed lung metastasis 2 years after operation, and brain metastasis was found in the same patient after 4 years. The recurrence occurred 3 years after OLT in another patient, no evidence of HAE recurrence or extrahepatic metastasis was found in the remaining 8 patients. Conclusions OLT can provide patients with advanced HAE the best chance for long term disease-free and overall survival who lost the opportunity for radical hepatectomy.