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The most important agents of endemic mycoses that can cause disease in healthy hosts are Histoplasma capsulatum, Blastomyces dermatitidis, Paracoccidioides brasiliensis and Coccidioides immitis. They occupy specific ecologic and geographic niches and exhibit temperature dimorphism. As these fungi are soil-based, people engaged in agriculture or recreational and work activities that expose them to disrupted soil are at the highest risk of infection. In Europe, only sporadic cases of autochthonic histoplasmosis were reported, but otherwise cases of endemic mycoses were reported only in patients from known endemic sites. This article discusses the characteristics of infectious agents causing endemic mycoses, along with their epidemiology and pathogenesis, as well as clinical manifestations, microbiological diagnostics and treatment of endemic mycoses.