Paracoccidioidomycosis is a systemic fungal infection caused by Paracoccidoides brasiliensis. The infection is endemic in subtropical areas of Latin America and has a high prevalence in Brazil. The disease is acquired by airborne inhalation of conidia and is frequently observed in adult male rural workers. The juvenile type of this mycosis is less prevalent (5-10% of clinical cases) and attacks both sexes. This clinical form occurs in children and adolescents and has a subacute course with fever, toxemia, loss of weight, adenopathy, hepatoesplenomegaly, anaemia and eosinophilia. Radiologic abnormalities in the lung fields may be seen. Mucous membrane lesions occasionally occur. The clinical presentation resembles severe tuberculosis, leukaemia or lymphoma. The diagnosis is confirmed by finding yeast-like elements of P. brasiliensis in microscopic examinations of wet preparations of specimens submitted for mycologic studies. The fungus grows slowly (20-30 days) and its isolation is difficult. Histologic and serologic studies may also assist in the diagnosis of this mycosis. Sulfonamides, ketoconazole, itraconazole, fluconazole and amphotericin B have been successfully used in the treatment of paracoccidioidomycosis. Itraconazole is the treatment of choice, being effective in more than 95% of cases. Co-trimoxazole is still frequently used especially in chronic progressive disease and as maintenance after a course of amphotericin B in severe cases of this mycosis.