Isolated intracranial Rosai-Dorfman disease (RDD) is extremely rare in pediatric patients. We present the case of a 22-month-old boy whom had isolated intracranial RDD involvement. To our knowledge, a parieto-occipital regional involvement without a dural tail sign has not been previously documented. Also, the mass contained hyperintense central T1 foci, and hypointense T2 and gradient echo foci; which are helpful in the differential diagnosis from meningioma. The magnetic resonance and computed tomography imaging findings are discussed and the follow-up course is presented in this paper.