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ABSTRACT Introduction Rosai–Dorfman disease (RDD) is an uncommon, benign, and idiopathic histiocytic proliferative disorder. Multiple intracranial RDD is extremely rare and treatment varies. Case presentation A 9‐year‐old girl was admitted with 3‐month history of blurred vision and facial paralysis, a 2‐month history of recurrent giggle, and cognitive impairment. Computed tomography and magnetic resonance imaging scans revealed bilateral ventricular masses based on the dural membrane and the diameters of the masses were 9.1 cm and 9.2 cm, respectively. The lesions were completely removed with staging surgeries. Fifteen months after operation, blurred vision was still present but facial paralysis and giggle and cognitive impairment disappeared. Imaging examinations suggested that there were no new or recurring lesions. Conclusion For multiple large intracranial masses, surgical treatment is necessary and staged surgery benefits perioperative safety. Active follow‐up with magnetic resonance imaging is necessary.