Histoplasmosis is a class of fungal disease mainly caused by Histoplasma capsulatum. Most of the immunocompetent people infected with Histoplasma capsulatum were self-limited and had few clinical symptoms. However, those with immunodeficiency, organ transplants and a history of travel in epidemic areas may suffer from disseminated histoplasmosis characterized by urgent onset, rapid development, and high mortality. Histoplasmosis was once considered to be an epidemic mycosis in the western hemisphere. Although it was regarded as a rare and imported infection in China, indigenous cases have been reported recently. To prevent misdiagnosis of the disease, epidemiological status, susceptible population, clinical manifestations, diagnostic methods and treatment progresses of disseminated histoplasmosis are reviewed in this paper.