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This report describes clinical and immunologic features of five illustrative cases of paracoccidioidomycosis in previously healthy children. All had disseminated disease and two of them died despite treatment. The major clinical presentation in four patients was fever and diffuse superficial and intraabdominal adenopathy, with or without hepatosplenomegaly. Other sites were also affected: three patients had multiple osteoarticular lesions, occasionally with intense tissue destruction; two had cutaneous eruptions; two had pericardial effusions; and two had pulmonary involvement, once considered an organ spared in the young. We detected variable lymphocyte responses to mitogens and toCandida albicansantigen and nonresponsiveness toParacoccidioides brasiliensiscell wall antigen. High concentrations of serum immunoglobulins and anti-P.brasiliensisantibodies were present. These immune alterations tended to resolve with treatment, suggesting a reversible nature of the immune defect. We conclude that this mycosis has a high morbidity and mortality in children, which is probably related to an antigen-specific immunodeficiency. Further studies are needed to increase knowledge of this mycosis in children.