Paragangliomas are rare benign neuroendocrine tumors derived from the extra-adrenal paraganglia of the autonomic nervous system. Here, we described a new case of a 75-year-old woman with paraganglioma arising in the middle and posterior cranial fossa with extended destruction of the skull base and clivus. She was admitted to our department with the complaint of severe respiratory distress due to vocal cord paralysis. We discussed its clinical, radiological, histopathological features and treatment modalities in the light of the current literature.
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