Rituximab (RTX) is a monoclonal antibody directed against the CD20 antigen expressed on B cells. It has been successfully employed in the treatment of non-Hodgkin's lymphoma and varied systemic autoimmune diseases such as rheumatoid arthritis, systemic lupus erythematosus, and granulomatosis with polyangiitis. Recently its efficacy in the treatment of ocular inflammatory diseases (OID), including refractory scleritis, peripheral ulcerative keratitis, uveitis, and ocular cicatricial pemphigoid, has aroused more concerns. The literature suggests that RTX may be useful for controlling the inflammation and decreasing or stopping the use of corticosteroids and other immunosuppressants in OID, which may contribute a new treatment alternative in patients with the recalcitrant and sight-threatening forms of OID. This article reviews the clinical application status of RTX in the treatment of OID.