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Rosai-Dorfman disease commonly involves cervical lymph nodes.Although extranodal involvement has been reported in diverse sites, central nervous system (CNS) manifestation, particularly in the absence of nodal disease is uncommon.We report 5 cases of RDD primary to the CNS without evidence of other sites of involvement.The cases included four men and one woman, ranging in age from 26 to 60 years , mean age was 41.2 years.The patients presented with headaches, seizures, numbness, or fever.Lesions in 3 cases were multifocal and located in the supratentrial and infertentorial area, 2 cases were separated located in the sellar region and left temporal lobe.Lesions were most often extra-axial and dura based.Histologically, the lesions consisted of variable numbers of pale-staining histocytes with emperipolesis often overshadowed by extensive lymphoplasmacytic infiltrates and fibrosis in the background.By immunohistochemical analysis, the characteristic histiocytes were positive for S100 protein and CD68 and negative for CD1a.Treatment consisted of surgical excision.