We focused on the epidemiology of alveolar echinococcosis especially in Japan and discussed control measures to prevent an epidemic. No effective control measures against alveolar echinococcosis have been identified thus far because it is difficult to fully understand the ecology of the parasite and its hosts, i.e. the precise infection route to humans. In Hokkaido, Japan, infection rates among red foxes have recently risen even in low endemic districts. Infection seems to be spreading not only among wild foxes but also among domestic dogs. Despite only sporadic reports of human cases in Japan, we predict that the incidence of alveolar echinococcosis will increase in the near future if no effective preventive measures are put in place. An Echinococcus multilocularis epidemic would have the potential to affect the economy of Hokkaido, due to its impact on the agricultural and tourist industries. Well-designed epidemiological surveys are, therefore, urgently required prior to large outbreaks, based on understanding of the ecosystem around E. multilocularis.