We herein report a case of retroviral rebound syndrome (RRS) complicated with hemophagocytic lymphohistiocytosis. Owing to the paucity of comprehensive data on RRS, we also conducted a literature review. All 19 cases included in the review presented within 2 months after the discontinuation of antiretroviral therapy. They were usually accompanied by both a significant decrease in CD4 count (median 292/μL) and a rapid increase in plasma human immunodeficiency virus loads (median 3.5×105/mL). Although life-threatening complications were reported, the overall prognosis was favorable. The outcomes of this review aided in the diagnosis of the present case.