Tabriz University of Medical Sciences Journal of Research in Clinical Medicine 2717-0616 8 1 2020 02 15 Isolated intracranial Rosai-Dorfman disease in an adult man: Report of a rare case 18 18 10.34172/jrcm.2020.18 EN Somaye Rezaei https://orcid.org/0000-0002-9311-815X Fariba Zarzanalivan Pouya Pirouti Mohammad Reza Amiri Nikpour Abdolreza Javadi Shahram Torkamandi https://orcid.org/0000-0002-8010-8286 Journal Article 10.34172/jrcm.2020.18 2020 04 04 2020 04 28 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.