The larval stage of Echinococcus multilocularis is the etiological agent of alveolar echinococcosis (AE), a parasitic zoonotic disease distributed in the Northern hemisphere, with an estimated 17 400 new infections/year, most of which occur in China. The life cycle of E. multilocularis involves small rodent intermediate hosts, such as arvicolids and, depending on the epidemiological settings, wild or domestic canid definitive hosts, such as red or arctic foxes, jackals, wolves, or dogs. Humans are aberrant intermediate hosts acquiring the infection through ingestion of eggs shed in the feces of definitive hosts. AE is a devastating clinical condition characterized by the silently progressing infiltrative proliferation of the parasite, mimicking a malignancy. AE is of increasing concern globally due to the geographical spread of the parasite, its increasing prevalence in animals from endemic areas, the absence of a vaccine, and the lack of active control measures to prevent the infection.