This systematic review and meta-analysis evaluated the effectiveness and safety of rituximab in patients with myasthenia gravis harboring antibodies to muscle-specific kinase (anti-MuSK). Four databases were searched from inception to December 23, 2023. We included adult patients (aged ≥ 18 years) who were diagnosed with anti-MuSK myasthenia gravis and who received rituximab. The outcomes assessed were the proportions of patients who achieved minimal manifestations or better and those who achieved complete stable remission or pharmacologic remission, according to the Myasthenia Gravis Foundation of America Postintervention Status (MGFA-PIS) scale at the last follow-up. Additional outcomes were mean glucocorticoid dose reduction and severe adverse events. Twelve studies with 111 participants were included. Overall, 82% (95% CI, 71‒91%; I2 = 30.12%, P = 0.15) of patients achieved MGFA-PIS minimal manifestations or better, and 56% (95% CI, 45‒67%; I2 = 0.00%, P = 0.60) achieved MGFA-PIS complete stable remission or pharmacologic remission. The mean reduction in the glucocorticoid dose was 17.15 mg (95% CI, 11.77‒22.53; I2 = 32.40%, P = 0.19). Only one patient developed osteomyelitis during rituximab treatment. This study demonstrated that rituximab is a safe and effective treatment for anti-MuSK myasthenia gravis, helping patients achieve minimal manifestations, complete stable remission, or pharmacologic remission with minimal serious adverse events.