Inflammatory myofibroblastic tumors of the orbit are uncommon lesions with unique histopathological features and uncertain pathogenesis. The authors report a case of an 8-month-old male infant who presented with approximately a 5-week history of left upper eyelid painless swelling that progressively grew in size. The patient underwent surgical debulking of the superior orbital mass through an upper eyelid crease incision. Review of the pathology slides revealed the tumor to be of myofibroblastic origin based on both histopathological and immunohistochemical examinations. Although the parents declined postoperative orbital irradiation or systemic corticosteroids, no recurrence was noted after 2 years of follow up.
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