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The authors describe a 9-year-old female who presented with swelling, proptosis, and tenderness of the right upper eyelid and MRI imaging demonstrating right lacrimal gland enlargement. After failing treatment with corticosteroids, the patient underwent a biopsy that was consistent with IgG4-related orbital disease. She was subsequently successfully treated with adalimumab (TNF-α inhibitor). This is the first case report of the successful use of a TNF-α inhibitor for the treatment of IgG4-related orbital disease in a child.