We report the case of a 16-year-old patient with osteopetrosis major, complaining of transient sensory and motor disturbances in the left upper limb and dizziness on changing the position of his head. Selective angiography of the cerebral vessels showed severe narrowing of the internal carotid artery within the petrous carotid canal and in its supraclinoid portion. The cervical vertebral arteries showed multiple stenosis within the vertebral canal. These findings are explained by narrowing of the basal foramina by the osteopetrotic bone.