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Extracranial meningiomas are rare. They account for only 2% of meningiomas compared to the much more common intracranial site. We describe a rare case of extracranial meningioma presenting as a neck lump in a patient with preexisting neck disease due to ankylosing spondylitis. Extracranial meningioma should be considered in the differential diagnosis of atypical neck lumps. Investigations including magnetic resonance imaging and histopathology are discussed, as well as the results of a literature review on extracranial meningiomas.