Rosai-Dorfman disease (RDD), also known as sinus histocytosis with massive lymphadenopathy, is a rare idiopathic histioproliferative disease affecting the lymph nodes. Although extranodal involvement has been reported in diverse sites, central nervous system manifestations, particularly in the absence of nodal disease with clinical and radiological findings suggestive of meningioma, are extremely rare. Histopathology and immunohistochemistry are essential for a positive diagnosis. We report a case of RDD in a patient presenting multiple meningeal nodules with a review of the literature and discussion of differential diagnosis.
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