The isolated intracranial Rosai-Dorfman disease (RDD) is extremely rare. It remains unclear for the best management of the residual lesions. In this report, the authors describe a case of primary single intracranial RDD treated with Gamma knife radiosurgery after partial resection, review all the related literatures and summarize the postoperative management data, in order to provide useful therapeutic information.A 23-year-old male presented with a more than 10-month history of dizziness, blurred vision and weakness. Preoperative MR imaging revealed a giant lesion involving the left middle cranial fossa and extending to the left cavernous sinus. He underwent fronto-temporal craniotomy and eventually achieved partial resection of the lesion. Postoperative histopathological results suggested a diagnosis of intracranial RDD. The residual lesion was treated with Gamma knife radiosurgery about 1 month after surgery. The follow-up imaging showed an obvious decrease in the size of the residual lesion.We are the second to provide the detail parameters of gamma knife therapy, which may be a reference for clinical neurosurgeons. Compared with the firstly reported case, our patient with a longer follow-up time has different therapeutic dose. Based on our own experience and an updated literature review, adjuvant stereotactic radiosurgery may be a safe and effective treatment for primary single intracranial RDD patients with incomplete excision. Other adjuvant therapies can be as an option in refractory patients.