| [Abstract]?Objective? The study is aimed to explore the clinical characteristics of hemophagocytic lymphohistiocytosis (HLH) in AIDS patients with opportunistic infections and malignancies. Methods?The prevalence, etiology, clinical features and prognosis of HLH among AIDS patients were studied retrospectively in Beijing Ditan Hospital from January 1, 2009 to December 31, 2021. Results?The prevalence of HLH in AIDS patients with opportunistic infections and malignancies and prevalence was 1.1%, with 18 cases of diagnosed HLH among 1641 AIDS patients. Penicillium Marneffei, Cytomegalovirus, EBV, tuberculosis, Mycobacterium avium complex (MAC), non-Hodgkin lymphoma and Kaposi’s sarcoma was the common causes of HLH. Ten cases (55.6%) died even after receiving corresponding treatment. Conclusions?Low prevalence and high mortality rate of HLH were found among AIDS patients with opportunistic infections and malignancies, which might be misdiagnosis and missed, further reminding doctors of occurrence of HLH among these patients.