OBJECTIVE The evidence from epidemiological research on whether the efficacy of rituximab in treatment of refractory nephrotic syndrome (NS) is better than other agents is inconsistent. This meta-analysis aimed to assess the efficacy of rituximab in the treatment of NS compared with other immunosuppressive agents. METHODS Relevant literatures were identified and evaluated for quality before October 2019 through multiple search strategies on PubMed and EMBASE. Statistical evidence of the symmetry of the funnel plot obtained from Begg's test was indicated by Egger's linear regression and a sensitivity analysis identified heterogeneity. A fixed- or a random-effects model was applied to calculate the pooled SMDs and RRs. RESULTS A total of 12 studies, involving 383 patients and 354 controls, were included. Compared with other agents, rituximab significantly improved complete remission both in children and adults [Overall: RR = 1.313, 95% CI = 1.170-1.475, P < 0.001; Adult: RR = 1.359, 95% CI = 1.053-1.753, P = 0.019 Children: RR = 1.354, 95% CI = 1.072-1.709, P < 0.001], and dramatically decreased the relapse rate in children [Overall: RR = 0.349, 95% CI = 0.166-0.732, P < 0.001; Children: RR = 0.286, 95% CI = 0.176-0.463, P < 0.001]. CONCLUSIONS Rituximab might be a promising treatment for refractory NS. Compared with other agents, rituximab significantly improves the complete remission and decreased the relapse rate. However, to confirm the efficacy of rituximab in the treatment of refractory NS, more high-quality, large sample, and multicenter randomized controlled trials are needed.