Case Report: A healthy 59-year-old male visited our hospital due to progressive bilateral temporal upper eyelid swelling for two years. On physical examination, elastic masses were palpable over temporal orbit without tenderness. The best corrected visual acuity of bilateral eyes was 6/6.7 on Snellen test. Intraocular pressure of right and left eye was 19 mmHg and 22 mmHg respectively. Extraocular movement revealed bilateral up gaze limitation and lateral gaze limitation of left eye. The computed tomography reported enlargement of bilateral lacrimal gland and left infraorbital nerve. Pathology diagnosis was compatible with IgG4-related orbital disease. The serum IgG4 level was 661 mg/dL. The patient received prednisolone 25 mg twice daily initially and tapered gradually. The mass decreased in size and also extraocular movement improved after steroid use. Conclusion: We conclude that orbital IgG4-related disease is a special entity of orbital inflammation. Systemic steroid use is an effective treatment.