Purpose This article focuses on understanding epidemiological features of alveolar echinococcosis and discussing its prevention and control, especially from a viewpoint of the ecosystem and risk management. Method Publications on alveolar echinococcosis throughout the world were systematically reviewed with special reference to ecology, epidemiology and countermeasures. Results Alveolar echinococcosis, caused by accidental infection with larva of the parasite Echinococcus multilocularis is fatal to humans unless diagnosed at an early stage. No effective control measures have been identified so far because it is difficult to fully understand the ecology of the parasite and its intermediate and definitive hosts. It is also not easy to determine the precise infection route to humans mainly because of the long latent period. In Hokkaido, infection rates among red foxes have recently risen even in low endemic districts. Not only stray and domestic dogs but also some pigs in Hokkaido have been found to be infected. While the number of reported human cases is still small, around 10 cases per year, local residents seem to be threatened with the risk of infection. Discussion and Conclusions We predict that the incidence of alveolar echinococcosis among humans in Japan will increase in the near future if no effective preventive measures are conducted. In addition, Echinococcus multilocularis infection has the potential to affect the economy of Hokkaido because of its impact on the agricultural and tourist industries. Well-designed epidemiological surveys are therefore urgently required, in the context of ecosystem and risk management prior to large outbreaks. International collaboration is also desired.