With the application of antiretroviral combination therapy (cART), the risk of acquired immunodeficiency syndrome-related lymphoma (ARL) has decreased, but it is still the most common acquired immunodeficiency syndrome (AIDS)-related tumor, in which B cells are the primary source. Human immunodefieiency virus(HIV)/AIDS-related diffuse large B-cell lymphoma (HIV/AIDS-DLBCL) is a subtype with the highest incidence, which is clinically associated with B-symptoms and involvement of extratodal organs. Chemotherapy regimen of cART combined with rituximab can significantly improve the chemotherapy tolerance and prognosis of patients with HIV/AIDS-DLBCL. This article discusses the epidemiology, pathogenesis and treatment strategies of HIV/AIDS-DLBCL, so as to further deepen clinicians′ understanding of HIV/AIDS-DLBCL.