Rosai-Dorfman Disease[RDD] is an idiopathic histiocytic proliferation affecting lymph nodes. Although extranodal involvement has been reported in the skin, orbit, upper respiratory tract, of testes, the isolated intracranical involvement without associated lymphadenopathy is extremely rare. We report our experience with 1 case of an isolated intracranial RDD without associated lymphadenopathy and any other organ involvement. A 61-year-old male presented with an isolated well-circumscribed brain mass in the posterior fossa, preoperatively thought to be a meningioma. But histology and Immunohistochemistry confirmed that the lesion was RDD.