Echinococcus multilocularis is a fatal zoonotic parasite in the Northern Hemisphere. Recently, it has become endemic in many countries in Asia, especially in the northern island of Hokkaido in Japan. The increasing threat of public health due to alveolar echinococcosis has compelled researches for sensitive diagnosis and effective control. This paper reviews on the epidemiology, diagnosis and control of echinococcosis specifically in Japan. International collaborative responses by researchers and government initiatives such as mandatory reporting system for veterinarians who diagnose echinococcosis in dogs are presented. Successful control measures in Japan using anthelmintic fortified baits for foxes are described. Assessment of prevalence rates during control campaigns is analyzed favoring the use of intravital diagnosis rather than the traditional necropsy method from hunting or trapping activities of wild foxes. The novel concept of "endogenous development" by local resident volunteers towards sustainable control of echinococcosis is stressed.