Background Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare non–Langerhans cell reactive histiocytic disorder that rarely occurs in the central nervous system (CNS). Extranodal RDD most frequently involves the skin, upper respiratory tract, soft tissue, gastrointestinal tracts, bones, breast, and CNS. Case Description RDD of the CNS infiltrates most commonly the dura of the sella, cavernous sinus, and the periclival regions. It is usually clinically and radiologically mistaken for meningioma because of its focal dural-based aspect. RDD is confirmed histologically by lymphoplasmacytic cells and histiocytes of varying size showing emperipolesis (lymphocytophagocytosis). To date, only 4 cases of RDD displaying spinal cord compression secondary to craniocervical junction involvement have been reported. Conclusion We report the case of a patient diagnosed with RDD localized at the foramen magnum extending to the base of the odontoid process and involving the V4 segment vertebral artery.
