Echinococcus ortleppi is the genotype G5 of Echinococcus granulosus sensu lato and is a zoonotic canine tapeworm of which larvae causes cystic diseases in domestic animals and also humans. While this species is highly endemic and widely spread in domestic animals, human infection is extremely rare and only sporadic, and thus, entire picture of human cystic echinococcosis due to infection with E. ortleppi is unclear. We have made an extensive literature review on the cases of E. ortleppi infection in humans and found a total of 19 cases from various places in the world with recent emergence in Asian countries.