Rosai-Dorfman disease (RDD) is a benign condition characterized by abnormal proliferation of white blood cells called histiocytes and an uncommon biological process called emperipolesis, in which a living cell penetrates inside another living cell. Central nervous system involvement in RDD is extremely rare. We report one such rare case of 47-year- old man who presented with right sided focal seizure from 1 year. MRI Brain showed a dural based lesion. On the basis of clinical presentation and radiological findings initial diagnosis made was of en plaque meningioma. Gross total resection of lesion was performed. Initial histopathological diagnosis was of IgG4 related HPM. Post surgery there was significant improvement in patients clinical condition. Intensity of seizure episodes were reduced. However due to persistence of right sided focal seizures, tissue blocks were reviewed and diagnosis of intracranial Rosai-Dorfman disease was made. Upon which further hematologist opinion was taken and whole body PET CT was done which showed solitary uptake in brain. It was observed that characteristic feature of RDD emperipolesis is sometimes masked by storiform fibrosis and lymphocytic infiltration leading to misdiagnosis. Therefore close follow up is required, in case of persistence of symptoms tissue blocks should be reviewed and possibility of intracranial Rosai-Dorfman disease should be considered while dealing with dural based lesions.
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