Abstract
Immunoglobulin G4 (IgG4)-related disease is a recently recognized inflammatory condition characterized by the infiltration of IgG4-positive plasma cells in various tissues, often presenting with systemic manifestations. While predominantly observed in adults, cases in pediatric patients are rare. This report details an unusual case of dacryoadenitis in an 8-year-old girl, who presented with a left superior-temporal orbital swelling unresponsive to initial treatments. Comprehensive clinical, radiological, and histopathological evaluations confirmed the diagnosis of IgG4-related dacryoadenitis. The patient was successfully treated with systemic corticosteroids, followed by the introduction of a steroid-sparing agent due to side effects. This case highlights the importance of recognizing IgG4-related disease in pediatric populations, emphasizing the need for a thorough diagnostic approach and tailored management strategies to improve outcomes and minimize complications.