Macrophage activation syndrome (MAS) is a serious complication of autoimmune diseases, with a potentially fatal risk, belonging to secondary hemophagocytic lymphohistiocytosis (HLH), often secondary to juvenile idiopathic arthritis (sJIA), adult still disease (ASOD), and can also be secondary to systemic lupus erythematosus (SLE), etc., SLE combined with MAS has a very high mortality rate, and the clinical manifestations are not easy to control high fever, pancytopenia, hepatosplenic lymphadenopathy, coagulation dysfunction, etc. Because SLE patients can have a variety of clinical manifestations, MAS is often difficult to identify, some patients are critically ill, and traditional glucocorticoids, immunosuppressants and other treatments are often difficult to control the disease, and the treatment of cytokines is gradually tried. This article reports a case of refractory SLE complicated with MAS successfully controlled by tocilizumab, and summarizes and analyzes the literature to explore the diagnosis and treatment experience of SLE complicated with MAS.