A 6-week-old-boy presented with a 3-week history of right axial proptosis. Vision, motility, anterior segment, and fundus examinations were normal in both eyes. Imaging revealed a multicystic right orbital lesion with extensive involvement of the infratemporal fossa and paranasal sinuses with intracranial extension. Systemic workup was negative, and he showed no functional deficits. Histopathology revealed a tumor rich in histiocytes, and immunohistochemistry indicated a juvenile xanthogranuloma. He did well with observation, and the tumor partially involuted after 18 months of follow-up.
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