Echinococcosis is a helminthic infection caused by ingestion of the eggs of the parasitic metacestode of one of four members of the Echinococcus genus. Among them, hepatic alveolar echinococcosis (HAE) mimics slow growing liver tumour with subsequent invasion to liver tissues and metastatic dissemination. Liver transplantation (LT) remains as the only treatment option when the lesions exceed the resectability limits. We described the first three cases of HAE who underwent orthotopic LT in Iran. A literature review was also conducted to summarize experiences of different transplant centres around the world.