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Introduction: Histoplasmosis is a rare mycosis with nonspecific clinical manifestation and a high misdiagnosis rate, which is an epidemic disease that occurred in several endemic areas. Cases of histoplasmosis were sporadic in China. Objective: We detected the clinical characteristics and endemic trend of histoplasmosis to understand it more profoundly. Method: The clinical data of 34 cases of histoplasmosis hospitalized in Xiangya Hospital of Central South University from 2003 to 2016 were analyzed retrospectively. The relevant literature were reviewed roundly which were about histoplasmosis from 2001 to 2019 reported in the Chinese mainland. Results: Thirty‐four patients were included in the study, male predominance. About 44.1% were combined with underlying disease and HIV infection was observed in eight patients. Common clinical manifestations were fever (94.1%), followed by lymphadenopathy (70.6%) and anemia (67.6%). Organ involvement included lungs (52.9%), oral cavity (5.9%) and intestine (5.9%). Patients were diagnosed by bone marrow smears (44.1%), lymph node biopsy (41.1%), lung tissue biopsy (8.8%) and intestinal tissue biopsy (5.9%). About 70.6% received amphotericin B, while 38.2% received itraconazole therapy and 79.4% were improved. About 611 cases of histoplasmosis in the Chinese mainland were obtained in the study from the related literature, with 217 (37.0%) cases occurred in Yunnan province, 69 (11.9%) cases in Hunan province and 67 (11.6%) cases in Hubei province. All of them were high incidence area of histoplasmosis. Conclusion: Histoplasmosis is a rare mycosis with high diagnosis rate and has a prominent geographical distribution in China, which should be considered highly in clinical work. [ABSTRACT FROM AUTHOR]