Purpose: We report a patient with refractory scleritis effectively treated with rituximab after ineffective, initial, conventional immunosuppressive therapy.Case summary: A 58-year-old woman with no specific medical history presented with recurrent scleritis of the right eye. Her uncorrected visual acuities in both eyes were 20/20, and a blood test for cytoplasmic antineutrophil cytoplasmic antibody (C-ANCA) was positive. Systemic and subconjunctival steroid treatments were not effective, and the scleritis recurred even after further treatment with oral cyclosporin A and mycophenolate mofetil. After intravenous administration of infliximab (5 mg/kg), slight improvement was noted, but the recurrence continued. Thereafter, the visual acuity of the right eye decreased markedly because of choroidal and serous retinal detachment caused by posterior scleritis. Immunohistochemically, the inflamed scleral tissue showed considerable B-cell infiltration. Accordingly, intravenous rituximab (1 g) was administered twice at a 2-week interval. The scleral vessel engorgement and inflammation dramatically improved, and the scleritis became well-controlled without recurrence for more than 8 months.Conclusions: Intravenous rituximab may be helpful when encountering scleritis refractory to conventional immunomodulation therapy.