Human alveolar echinococcosis, caused by the metacestode stage of Echinococcus multilocularis, is one of the most dangerous zoonoses in the temperate and arctic areas of northern hemisphere. The mortality of the disease exceeds 95% in untreated or inadequately treated patients. In the past three decades, the spread of this parasite was observed in Europe as a consequence of human interventions resulting in the population size increase of foxes. The authors demonstrated the presence of E. multilocularis in foxes in all northern counties of Hungary and the existence of hyperendemic regions in the Counties Nógrád and Gyor-Moson-Sopron. The aim of the present paper is to summarize the current knowledge on the life cycle and distribution of E. multilocularis, and the epidemiology, clinical features, diagnosis, treatment and control of human alveolar echinococcosis.