An aberrant internal carotid artery in the middle ear is a potentially disastrous anatomical variation. An accurate diagnosis of
this anomaly is essential to prevent catastrophic complications such as massive hemorrhage during otologic procedures. Any
intervention on the middle ear without the evaluation of this variation could be fatal, so tomography and magnetic resonance
could be very useful to identify this rare entity and to avoid its complications. We report a case of an aberrant internal carotid
artery appeared as pulsatile unilateral tinnitus.