We report the case of a 41-yr-old woman who presented with arterial hypertension and tinnitus in the right ear synchronous with pulse. She had previously undergone surgery for suspected pheochromocytoma without positive therapeutic effect. CT and MRI revealed a homogenous tumor with contrast enhancement in the right hypotympanon and foramen jugulare, and [123I]metaiodobenzylguanidine (MIBG) scintigraphy demonstrated strong tracer uptake in the same area. Selective venous sampling of catecholamines in the ipsilateral jugular vein confirmed the tumor to have originated from hormone production.