The authors present a case of necrotizing scleritis after pterygium excision successfully treated with rituximab after attempts with high doses of corticosteroids and immunosuppressive drugs. A literature review revealed case reports and a phase I/II dose-ranging randomized clinical trial using rituximab for necrotizing scleritis with or without association with autoimmune disease. This is the only case report on rituximab treatment for necrotizing scleritis after pterygium surgery. In cases with refractoriness to immunosuppressive drugs, a CD20 antibody can be used.