A 15 year-old-male presented with concurrent sinonasal polyposis and anterior uveitis with cervical lymph node enlargement. Cervical lymph node biopsy confirmed Rosai-Dorfman disease. Though patient's eye symptoms did not respond well to topical corticosteroid treatment but he showed a remarkable regression in the size of nasal polyps and cervical lymph nodes after systemic corticosteroids. Intraocular involvement in such a case is rare.
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