Meningiomas are tumours typically derived from the meningothelial cells of the arachnoid mater. They most often arise in intracranial, intraspinal, or orbital locations. Ectopic meningiomas, described as primary meningiomas with no intracranial involvement, are definitely unconventional. In fact, most of the extracranial meningiomas described in the literature, particularly in the outer ear, are effectively spreads of disease with primary intracranial localization. We describe a case of a primary external auditory canal meningioma with demonstrated absence of intracranial involvement, and we provide a full radiological, histological, immunohistochemical and molecular characterization of the lesion.
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