A healthy 52-year-old male (J.L.A.) experienced a right pulsatile tinnitus (PT). It was synchronous with cardiac systole, was noticed initially at night, and became objectively audible. Valsalva’s maneuver markedly diminished its intensity. There were no previous trauma, otalgia, hypoacusis, fever, nasal congestion, cough, headache or any neurological symptom. Arterial pressure, ECG, chest X-ray, neuro-otological examination, otoscopy, audiometric testing, tympanometry and cerebral magnetic resonance (MR) imaging were unrevealing. A cerebral magnetic resonance angiography (MRA) raised the suspicion of a possible dissection of an extracranial right internal carotid artery (ICA) near the skull base. Subsequently, a cervical CT angiography (CTA) and a cervical MR angiography (MRA) yielded the diagnosis of right steno-occlusive (less than 50% luminal stenosis) ICA dissection, extending from 1 cm above the carotid bulb to the cranial base, ending just proximal to the petrous carotid canal. The dissection was associated with a medium-sized saccular pseudoaneurysm located near the skull base—C1-C2 level. Digital subtraction angiography (DSA) confirmed the diagnosis and showed bilateral fibromuscular dysplasia of carotid arteries as the cause of the dissection (Fig. 1). No intravascular intervention (i.e., stenting) was considered necessary due to the high location of the pseudoaneurysm and the lack of neurological symptoms. Treatment with intravenous heparin was followed by aspirin. Tinnitus gradually disappeared in 4 days. At follow-up, CT demonstrated resolution of the luminal stenosis and thrombosis of the false lumen, although the pseudoaneurysm remained unchanged.