Objectives: Mucormycosis is an invasive fungal infection afflicting immunocompromised patients, causing a significant degree of morbidity and mortality. The purpose of the study was to provide a comprehensive analysis describing the epidemiology and outcome of mucormycosis in the scenario of HIV infection. Methods: We systematically searched PubMed for reports about mucormycosis associated with HIV. Eligible studies describe the predisposing factor, clinical form, treatment, and survival outcome. Results: We included 61 articles from 212 reviewed abstracts, corresponding to 67 cases. Patients were mostly men (68.2%) with a median CD4(+) count of 47 [IQR 17-100] cells/mm(3). Intravenous drug use (50%), neutropenia (29.7%) and corticosteroid use (25%) were the predominant associated factors. The main clinical forms were disseminated (20.9%), renal (19.4%), and rhino-cerebral (17.9%). Rhizopus (45.5%) and Lichtheimia spp (30.3%) were the main fungal isolates. Treatment consisted of antifungal therapy and surgery in 38.8%. Overall mortality rate was 52.2%, and varied with the site of infection: 92.9% for disseminated disease, 62.5% for cerebral disease, 60% for pulmonary infection, and 36.4% for cutaneous infection. Survival was worse for those who did not initiate antifungals (p = .04), who were antiretroviral naive (p = .01), who were admitted to ICU (p = .003) or had disseminated disease (p = .007). Conclusions: Mucormycosis is a life-threatening infection in HIV patients and clinician should be aware of this co-infection in the differential diagnosis of HIV opportunistic infections. (C) 2016 The British Infection Association. Published by Elsevier Ltd. All rights reserved.