Disseminated histoplasmosis is a disease with a high case-fatality rate, especially in patients with the acquired immunodeficiency syndrome (AIDS). The disease can occur in various sites, such as the lungs, eyes, oral cavity, larynx, nervous system, gastrointestinal tract and, more rarely, the nasal sinus region. It is a cosmopolitan mycosis with a high prevalence in Brazil. Nasal manifestation of the disease is rare, with only three cases reported in the literature, but it is part of the differential diagnosis for other granulomatous diseases, such as Wegener's granulomatosis, tegumentary leishmaniasis and nasal lymphoma. The authors of this study present a literature review and report a case of nasal histoplasmosis in a patient with AIDS. No record of such an aggressive presentation has been reported previously in the literature.