Background and Aims Paracoccidioidomycosis is the most frequent systemic mycosis in Latin America and mainly affects male adults, with a past history of working in rural areas, presenting with chronic pulmonary and mucosal lesions. However is rare in children and the clinical presentation is quite different, seemingly mostly with lymphoma or disseminated tuberculosis. The aim of this study is describe the clinical presentation, evolution and response to treatment of three cases of paracoccidioidomycosis affecting children, living in a city of Rio de Janeiro state, Brazil.

Methods Revision of the clinical charts of children who were admitted in a general pediatric ward of a teaching hospital, with confirmed diagnosis of paracoccidioidomycosis.

Results During a period time of four years, there children (two boys and one girl), were admitted with a subacute clinical picture of a generalized lymphoadenopathy (mainly cervical), fever, weighting loss, anemia, with had clinical diagnosis of lymphoma.

There was no apparent pulmonary or mucosal lesions and abdominal ultrassonography showed multiples lymphoadenopathies. Biopsy of cervical lymphonode showed Paracoccidioides brasiliensis. They were initially treated with amphotericin B and then, followed by oral itraconazol (2 cases) and ketoconazol (1 case) for one year. All had a excellent response to treatment and are being followed at the out patient clinic of infectious diseases, without relapse.

Conclusion In Latin America, Paracoccidioidomycosis should be included in the differential diagnosis of lymphoma and tuberculosis in children presenting with subacute lymphoadenopathy and biopsy looking for fungal forms is essential to establish the diagnosis.