Abstract: OBJECTIVE To evaluate the efficacy and safety of rituximab(RTX) therapy on pediatric refractory nephrotic syndrome (NS). METHODS According to the selection criteria and elimination criteria, retrieved relevant databases and obtained literatures about RTX in the treatment of pediatric refractory NS. The extractive data(serum albumin, serum creatinine, proteinuria) was performed statistical analysis. The effects of RTX on remission rate and relapse-free survival rate of pediatric refractory NS were meta-analyzed. RESULTS A meta-analysis of complete remission rate of pediatric refractory NS treated with RTX showed that RTX treatment group was significantly better than that in control groupOR=4.15, 95%CI(1.80, 9.57), P<0.01. RTX therapy could improve the serum albuminWMD=0.46, 95%CI(0.00, 0.92), P=0.05 and reduce the urinary proteinWMD=-0.25, 95%CI(-0.29, -0.21), P<0.01 in pediatric refractory NS. However, there was no significant difference in serum creatinine level(P=0.89). Meta-analysis suggested that there was a significant difference in relapse-free survival rateOR=0.43, 95%CI(0.31, 0.61), P<0.01. CONCLUSION RTX therapy can improve the complete remission rate and relapse-free survival rate, which is benefit to long-term prognosis in pediatric refractory NS.