Background: To assess the efficacy and safety of rituximab (RTX) in the treatment of neuromyelitis optica spectrum diseases (NMOSDs), and give a guideline on clinical medication Methods: The databases of Pubmed, Embase, Cochrane Library, CNKI, and Wan fang were systematically searched by computer, and the search period was from the establishment of the databases until January 2022. To collect the trials of RTX in the treatment of NMOSDs, two researchers completed literature screening, quality assessment, and data extraction independently. Statistical analysis was performed using Review Manager 5.3 and Stata 15.1 software.Results: There were 37 studies in the meta-analysis, including 5 randomized controlled trials (RCTs) and 32 observational studies. Meta-analysis results revealed that NMOSDs patients treated with RTX significantly reduced the annualized relapse rate (ARR) (weighted mean difference [WMD] = 1.45, 95% confidence interval [CI]: 1.24-1.66, P < .01) and the Expanded disability status scale (EDSS) scores (WMD = 1.34, 95%CI: 1.25-1.44, P < .01). RTX is more effective than azathioprine (AZA) in the treatment of NMOSDs