Paracoccidioidomycosis (formerly known as South American blastomycosis) is produced by the thermally dimorphic fungus Paracoccidioides brasiliensis. Most often this mycosis runs a chronic progressive course affecting preferentially the lungs followed by the skin, mucous membranes, adrenals, and reticuloendothelial organs. Acute-subacute presentations can be observed in children and immunosuppressed patients. Occasionally, self-limited infections have been documented. Two types of clinical presentations are described, the acute-subacute (juvenile) and the chronic (adult) forms of the disease. Paracoccidioidomycosis predominates in adult males (13:1); this gender difference is not observed in children or adolescents. The mycosis is limited geographically to various Latin American countries, with the greatest number of cases originating in Brazil, The fungus's natural habitat has not been precisely defined, although it is supposed to be a soil-inhabiting microorganism. No outbreaks have been reported. P. brasiliensis is capable of entering into prolonged periods of latency as is demonstrated by its diagnosis in patients who have moved outside the recognized endemic areas. This review updates clinicians and laboratory workers on the characteristics of a mycosis seldom reported outside of the Latin American countries.