Abstract
Background: Isolated intracranial Rosai-Dorfman disease (RDD) is an extremely rare, idiopathic histo-proliferative disorder. RDD is associated with the proliferation of histiocytes and emperipolesis.
Case Presentation: we report a case with isolated intracranial RDD. A 47-year- old man presented with a dizziness, falling, and then secondary generalized seizure, hemiparesis and right hemisensory deficit. This case preoperatively was misdiagnosed with meningioma. Histopathological examination revealed pale histiocytes displaying emperipolesis which were positive for S-100 and CD68 proteins and negative for CD1a marker. BRAF V600E mutation was negative.
Conclusion: In this case, total resection was performed and clinical symptoms were regressed completely.