Hokkaido is the northernmost island and the only endemic area of alveolar echinococcosis caused by Echinococcus multilocularis in Japan. The first human case was reported in 1937. After the discovery of this disease, various epidemiological surveys have been conducted on Echinococcus multilocularis in animals. Accumulated longitudinal epidemiological data clearly showed the geographic changes of endemic areas in Hokkaido during the last 70 years. The expansion of the endemic area and the increase of the prevalence of the parasite in foxes have increased the risk of infection for residents in this region during the last two decades. This paper reviews the epidemiological situation and control options of this parasite in animals after a brief description on the historical background of this disease in Hokkaido.