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Rosai Dorfman disease is a rare histiocytic disorder. It is also known as Sinus Histiocytosis. It is with massive lymphadenopathy involves an overproduction of a type of white blood cell. The disease is rarely associated with intracranial and intraorbital involvement. Intracranial Rosai-Dorfman can mimic meningioma. Other pathologies also underline its pathologies. Here, we report a nine-year-old boy with a history of proptosis of the right eye and presenting with multiple skull lesions. Histopathological study revealed Sphenopetroclival lesion, which features that of Rosai Dorfman Disease. His MRI scan of the brain was taken, which showed evidence of right optic nerve meningioma with sella and suprasellar extension, causing severe proptosis. The child underwent right frontotemporal craniotomy with petrosectomy and Transylvanian, subtemporal approach to multicompartmental Rosai-Dorfmans lesion. After four months, the patient had a recurrence of the disease on which chemotherapy and steroids were started, which also did not show much response while taking an MRI scan. A corticosteroid is a useful option in the Central Nervous System Rosai Dorfman disease treatment. But this patient showed a negative outcome to the treatment.
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