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Introduction In this prospective, open‐label study we explore the effectiveness of low‐dose rituximab every 6 months in treating refractory generalized myasthenia gravis (GMG). Methods Twelve patients with acetylcholine receptor (AChR)‐positive refractory GMG were enrolled for the study. The primary endpoint was the change in quantitative myasthenia gravis (QMG) score from baseline to the study end. Secondary endpoints included changes in manual muscle testing (MMT), MG‐Related Activities of Daily Living (MG‐ADL), and 15‐item Quality‐of‐Life (MGQOL‐15) scores, as well as prednisolone reduction. Results MG decreased from 18.25 ± 4.03 to 8.42 ± 3.99 (P = .0001), MMT from 27.50 ± 17.78 to 4.58 ± 4.34 (P = .0001), ADL from 8.50 ± 2.84 to 1.17 ± 1.27 (P < .0001), MGQOL‐15 from 37.25 ± 13.78 to 17.50 ± 9.73 (P = .0015), and prednisolone dose from 29.38 ± 11.92 mg/day to 8.86 ± 1.88 mg/day (P ≤ .01). Discussion Low‐dose rituximab every 6 months is effective in treating refractory GMG patients.