Clinical and epidemiological features of 422 cases of paracoccidioidomycosis attended at University Hospital of Universidade Federal de Mato Grosso do Sul (Campo Grande, Mato Grosso do Sul, Brazil) from January 1980 to August 1999, were analysed. The mean age was 43.4 years old and the male: female ratio was 10:1. Nearly half (45.5%) of the patients were agricultural workers at the moment of diagnosis. In the acute/subacute form (juvenile type) the phagocytic-monocytic system was very much impaired and mainly marked by lymphadenopathy (95.4%), hepatomegaly (40%), splenomegaly (23.1%). The chronic form (adult type) presents more lesions in oropharynx (66.4%), dysphonia (31.4%) and cough (50.7%). Mycological diagnosis was obtained by direct microscopy of wet mounts in 185/365 (50.7%) patients and by histopathological examination of biopsies in 294/302 (97.3%) patients. The treatment of choice was Sulfamethoxazole/ trimethoprim (Co-trimoxazole), used in 90.3% patients. Sequelae occurred in 30.3% and death in 7.6% of the cases.