We describe a patient with acquired immunodeficiency syndrome who developed candidiasis-associated hemophagocytic lymphohistiocytosis (HLH), and we review the previously reported cases of this unusual clinical syndrome in patients infected with human immunodeficiency virus (HIV). HLH appears to follow a fulminant course in HIV-infected patients, which warrants an aggressive diagnostic and therapeutic approach. HIV itself may play a role in the pathogenesis of HLH, which is usually associated with opportunistic infections or malignancies. Therapy is usually directed at supportive care and treatment of the underlying disorders, although initiation of antiretroviral therapy may improve the eventual outcome in some cases.