Rosai-Dorfman disease (RDD) is an abnormal proliferationof histiocytes which manifest classically as bilateral cervicallymphadenopathy and B symptoms. Rarely, it also presentswith involvement of other systems. CNS RDD is extremelyrare and accounts for 5% of reported cases. The clinicalpicture is dependent on the area of CNS affected. It ismostly diagnosed on MRI, however, it may be confusedwith a meningioma, dural based metastases, lymphoma,sarcoidosis, etc. Diagnosis is based on typicalhistopathological features. The recommended treatmentfor symptomatic CNS manifestations of RDD is completesurgical resection. Keywords: Rosai-Dorfman disease, Central NervousSystem, Histopathology