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A 12-year-old boy had central von Recklinghausen's neurofibromatosis with multiple intracranial meningiomas and bilateral acoustic neurinomas. One of the meningiomas was found in the parenchyma of the medulla oblongata and was successfully removed. To our knowledge, this is the first reported case of surgical removal of an intramedullary meningioma of the medulla oblongata. Details of the clinical, neuroradiological, and operative findings are described.