Purpose <p>To report a case of chronic unilateral nodular anterior scleritis as a rare, isolated presentation of IgG4-related ophthalmic disease.</p> Case <p>A fifty-two-year-old patient was evaluated for painful, nodular scleral injection of the right eye, diagnosed as nodular anterior scleritis. There was only a partial response to topical corticosteroid and systemic immunomodulatory therapy. This, in combination with a sharp delineation between normal and abnormal sclera lead to the decision to perform a diagnostic biopsy of the lesion. Histopathology showed an IgG4 lymphoplasmacytic infiltration of the scleral tissue.</p> Conclusions <p>There are few documented reports of scleritis as the presenting manifestation of IgG4-related disease. IgG4-related disease is an increasingly recognized etiology to be considered in evaluating and managing scleritis.</p>