We described a case of recurrent extranodal extra-and intracranial communicating Rosai-Dorfman disease.A middle-aged female patient was admitted in May 2017 with a recurrent scalp tumor after surgery.The mass lesion and the involved dura mater and skull were completely removed.The pathological diagnosis was Rosai-Dorfman disease(RDD).No lymphadenopathy was found all over the body.She took oral prednisone for one month.She was followed-up regularly and no recurrence was found.In December 2022,she had a nodular mass on the skin of the right forehead and was diagnosed as having RDD by biopsy.Because the oral administration of thalidomide was ineffective,the mass was resected totally.In October 2023,a follow-up MRI revealed a mass lesion involving the superior sagittal sinus and the cerebral falx in the right fronto-parietal area.The mass lesion was removed totally and the pathological diagnosis was RDD.There was no recurrence during eight-month follow-up.This report showed that RDD involving the central nervous system can recur repeatedly.Therefore,the long-term follow-up should be mandatory.Microsurgical resection of the extranodal extra-and intracranial communicating RDD was safe and effective.Reoperation can be performed in patients with recurrence.

We described a case of recurrent extranodal extra-and intracranial communicating Rosai-Dorfman disease.A middle-aged female patient was admitted in May 2017 with a recurrent scalp tumor after surgery.The mass lesion and the involved dura mater and skull were completely removed.The pathological diagnosis was Rosai-Dorfman disease(RDD).No lymphadenopathy was found all over the body.She took oral prednisone for one month.She was followed-up regularly and no recurrence was found.In December 2022,she had a nodular mass on the skin of the right forehead and was diagnosed as having RDD by biopsy.Because the oral administration of thalidomide was ineffective,the mass was resected totally.In October 2023,a follow-up MRI revealed a mass lesion involving the superior sagittal sinus and the cerebral falx in the right fronto-parietal area.The mass lesion was removed totally and the pathological diagnosis was RDD.There was no recurrence during eight-month follow-up.This report showed that RDD involving the central nervous system can recur repeatedly.Therefore,the long-term follow-up should be mandatory.Microsurgical resection of the extranodal extra-and intracranial communicating RDD was safe and effective.Reoperation can be performed in patients with recurrence.