Introduction: Patients with steroid-dependent nephrotic syndrome (SDNS) suffer frequent relapse with adverse effects caused by long-term prednisolone treatment. Recently, the chimeric monoclonal antibody against the protein CD20 (rituximab-RTX) was observed to be efficacious and safe in the treatment of patients with SDNS. We summarized the scientific literature to evaluate RTX therapy in the clinical management of SDNS. Material and methods: PubMed, EMBASE, and Cochrane Library databases were investigated from interception to 2019-6-6, without language limitation. The analysis was restricted to adults ≥ 19 years of age. Data were administered and analyzed through the Review manager 5.3 software. Results: After RTX treatment, relapse times, prednisolone dose, and proteinuria decreased, whereas serum albumin was increased. The clinical parameters blood pressure and total cholesterol diminished also, whereas bone mineral density was improved. Overall, RTX ameliorated the adverse effects of prednisolone. Moreover, the Th1/Th2 ratio was changed except for the CD19 and CD20 cell counts. Additionally, most of the adverse effects of RTX were mild and well tolerated. Conclusions: In the studies that we considered, we concluded that RTX treatment was effective and safe in the therapy of patients with SDNS. Nevertheless, more randomized controlled trials are required to explore the mechanism of RTX action and verify its efficacy.