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To report a case of isolated subconjunctival ocular adnexal IgG4-related disease that met the diagnostic criteria according to the Japanese Ministry of Health, Labour and Welfare's 2011 guidelines.We report a case of a 56-year-old woman with a left subconjunctival mass for 3 months. Excisional biopsy was performed. Postoperatively, the patient underwent systemic and radiologic evaluations for IgG4-related disease.The clinicopathologic study revealed storiform fibrosis and lymphoplasmacytic infiltration, with increased IgG4-positive plasma cells and an IgG4/IgG-positive plasma cell ratio of 40%. Serum IgG4 and IgG levels were slightly elevated. Systemic involvement was not detected.IgG4-related disease is well known in the orbit and ocular adnexa, particularly the lacrimal gland. However, subconjunctival involvement should be recognized as a possible presentation for this entity.