From 1977 to 1990, 139 patients with active paracoccidioidomycosis were diagnosed in the mycology laboratory of the CIB. Only 4(2 9 %) of them exhibited the juvenile form of the disease. The patients were all males, 5-14 years of age and presented important involvement of the reticuloendothelial system. The Initial clinical diagnosis was not paracoccidioidomycosis; In one patient the disease was diagnosed as non-Hodgkin Lymphoma; In another, as ganglionar TBC while In the remaining two, the diagnosis was not attempted. The multiple budding yeast cells of P. brasiliensis were observed in biopsy specimen (cervical lymph nodes) in two patients and in direct (KOH) examination (cutaneous and ganglionar lesions) in the remaining two. P. brasiliensis was demonstrated by direct (KOH) examination and by culture in sputum samples obtained from the four patients; this fact points towards the lungs as the organ of primary affection in this mycosis. The patients were treated with Imidazole derivatives and their outcome was evaluated according to a scoring system; the latter revealed that three patients had marked Improvement and one, complete resolution of the pre-therapy abnormalities. In the endemic areas, paracoccidioidomycosis should be taken into consideration in children with respiratory diseases and Lymph hypertrophy.