Cervical internal carotid artery fenestration (fcICA) is an extremely rare anatomic variation. Its embryological backround is merely a hypothesis and still not fully understood, causing many authors to question its existence and support the theory of an underlying pathologic mechanism such as dissection. Since distinction between ICA fenestration and pseudofenestration is usually not straightforward, patients should be examined carefully with all available imaging modalities to exclude potential subjacent arterial dissection. We present an interesting case of a 47-year-old woman with fcICA on the ground of fibromuscular dysplasia (FMD), who presented with ipsilateral pulsatile tinnitus.