Abstract: Background: Pediatric antimyelin oligodendrocyte glycoprotein antibody associated disease (MOGAD) has a variety of clinical manifestations, which can be divided into monophasic and polyphasic courses according to the course of disease, of which about 50% children present with a polyphasic course. Recurrent episodes of polyphasic MOGAD lead to varying degrees of neurological impairment. The main prophylactic drugs for relapse of multiphasic MOGAD include mecophenolate mofetil（MMF）, rituximab (RTX), azathioprine (AZA) and intravenous gamma globulin (IVIG) on a monthly basis. However, there is a lack of highquality clinical evidence for the prophylactic treatment. Objective: To investigate the effectiveness of rituximab in the treatment of MOGAD through a systematic review and metaanalysis. Design: Systematic review/metaanalysis. Methods: Literature on rituximab in the treatment of MOGAD was searched in the databases of Pubmed，Embase，Web of Science，Cochrane，Wanfang Data，CKNI and VIP from Jan 1st, 1997 to June 1st, 2021. After two researchers independently screened the literature, extracted the data, and evaluated the risk of bias in the included studies, the metaanalysis was performed using RevMan 5.3. Main outcome measures: Annualized relapse rate and relapsefree rate. Results: Thirteen studies (272 patients) were included. Metaanalysis showed that the relapsefree rate of patients of MOGAD who received rituximab treatment was 32.4%（95%CI:2.9%47.9％，I2=52%，P=0.03）, and the annualized relapse rate changes -1.44（95%CI: -1.67-1.21，I2=71%，P=0.004）. Conclusion: Rituximab reduced the frequency of relapse in some MOGAD patients, but the relapsefree rate was only32.4%. The results of this study need to be verified by more highquality studies.

Key words: Anti-myelin oligodendrocyte glycoprotein-antibody associated disease, Immunotherapy, Rituximab, Meta-analysis