Histoplasmosis occurs in specific endemic areas, including the mid-western United States, Africa and most of Latin America. Sporadic cases have also been reported in China. The aim of this study was to summarise the epidemiological and clinical data of histoplasmosis in China. We searched the PubMed, CBMdisk and CNKI databases to identify publications related to histoplasmosis in China. Case reports/series on patients with histoplasmosis were included. A comprehensive literature review identified additional cases. The relevant material was evaluated and reviewed. Overall, 300 cases of histoplasmosis were reported in China from 1990 to 2011, and 75% were from regions through which the Yangtze River flows. Most of the patients were autochthonous infections. Of these, 43 patients had pulmonary histoplasmosis and 257 patients had disseminated histoplasmosis. Common underlying diseases included HIV infection, diabetes mellitus and liver diseases. Fever was the most frequently reported clinical feature in disseminated histoplasmosis, followed by splenomegaly and hepatomegaly. Cases of histoplasmosis had a prominent geographical distribution in China. Histoplasmosis should be considered in the diagnosis of patients with relevant symptoms and a history of travel to or residence in these areas.