The endemic mycoses are a group of infections caused by fungi with a distinct geographic distribution, defined by climatic and environmental conditions. The systemic endemic mycoses, namely histoplasmosis, blastomycosis, talaromycosis, coccidioidomycosis and paracoccidioidomycosis, occur after the inhalation of fungal spores. The cutaneous endemic mycoses, including sporotrichosis, mycetoma, entomophthoramycosis and chromoblastomycosis, enter the host via traumatic inoculation of the skin. Clinical presentation varies between these relatively heterogeneous infections, as does the susceptibility of immunosuppressed patients to disease. An understanding of the geographic range, typical manifestations, diagnostic methods, and treatment of the endemic mycoses is key in assessing patients presenting with atypical infections who may have traveled to endemic areas.