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Introduction: Disseminated histoplasmosis (DH) is a systemic mycosis caused by Histoplasma capsulatum (H. capsulatum) and is characterized by progressive and fatal evolution in immunocompromised patients. Moreover, it is considered an AIDS-defining disease., Methods: We performed an observational, analytical, retrospective study to identify the clinical outcomes and risk factors for death from DH in patients with AIDS at an infectious diseases service facility in Brazil between September 2011 and July 2016. Patients with a positive serology for HIV and DH were diagnosed via direct examination and/or positive cultures for H. capsulatum., Results: Twenty-three patients were included in this study. Approximately, 82.6% were men, with a mean age of 41.0±11.5 years, and 52.2% had a concomitant diagnosis of AIDS and DH. The median CD4+ T cell count was 19 cells/mm3, and 56.5% of the patients died. The most frequently observed symptoms were fever, dyspnea, and skin lesions. On the basis of a comparative analysis of those who died and survived, the absence of splenomegaly and hepatomegaly and the presence of H. capsulatum in the peripheral blood were considered as risk factors for death. Those who died had a higher leukocyte count; CRP, urea, and lactate dehydrogenase levels; AST index; and international normalized ratio prothrombin time. The serum total protein and albumin levels of the patients were lower., Conclusions: The mortality rate for DH is high among severely immunocompromised patients with AIDS. The risk factors for death were those traditionally associated with blood dyscrasia, inflammatory activity, as well as increased renal and nutritional impairment.