Encephalitzoon spp. are microsporidia, and intracellular opportunistic pathogens. The hosts of these pathogens include vertebrates, invertebrates, and certain protozoa. In people microsporidia may be opportunistic pathogens for immunocompromised patients (with AIDS or after organ transplantation). Infection with these microorganisms was also described in persons with diarrhea and corneal diseases.The species causing rare infections in humans, Encephalitozooncuniculi, had previously been described from animal hosts. However, several new microsporidial species, including E. intestinalis and E. hellem, have been discovered in humans, raising the question of their natural origin. Vertebrate animals are now identified as hosts for all three microsporidial species infecting humans, implying a zoonotic nature of these microorganisms. Molecular studies have identified phenotypic and/or genetic variability within these species, indicating that they are not uniform, and have allowed the question of their zoonotic potential to be addressed. The focus of this review is to present the zoonotic potential of E. intestinalis, E. cuniculi, and E. hellem