Objective To investigate the clinical manifestations,imaging,clinicopathological,immunohistochemistry,treatment and prognosis of intracranial primary Rosai-Dorfman disease(RDD).Methods The clinical data of a patient with intracranial primary RDD admitted to The First Affiliated Hospital of Xinjiang Medical University were analyzed retrospectively,and the relevant literature were reviewed.Results The magnetic resonance imaging of the head showed an occupying lesion on the left parietal temporal area,and surgery was performed to completely remove the lesion.Postoperative pathology was diagnosed as RDD,immunohistochemistry showed S-100(+),CD68(+).Conclusions RDD is a benign disease,and its diagnosis is mainly based on pathology and immunohistochemistry.Surgical resection is currently the most effective treatment for RDD.For multiple or unresectable lesions,radiotherapy and chemotherapy can be given as an adjunct.