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Carotid body tumors (CBTs) are rare hypervascular lesions with critical location which makes them very challenging to treat. In rare occasions, compression of the jugular vein from the tumor mass could predispose to progressive thrombosis of intracranial venous sinuses. The latter consequently leads to intracranial hypertension (pseudotumor cerebri) with the accompanying danger to the vision. Herewith, we present our management strategy for this rare presentation of CBTs. A 38-year-old woman, with no medical history, was admitted in the em