Absence of the vertebral artery is rare, and incidentally encountered in radiological imaging technics. We reported a 74 years old man suffering from pulsatile tinnitus with absence of the left vertebral artery. The purpose of the case report is the description absence of the vertebral artery causing of pulsatile tinnitus, in order to offer useful data to anatomists, otorhinolaryngologist, radiologists, vascular, head and neck surgeons.