Vasospasms of the intracranial arteries are a well-known complication of subarachnoid hemorrhage and are also frequently encountered in other disorders such as migraine, cerebral vasculitis or reversible cerebral vasoconstriction syndrome. In contrast, recurrent spontaneous vasospasms of the extracranial circulation appear to be extremely rare and have most often been associated with migraine. We present a patient with recurrent strokes due to spontaneous transient vasospastic occlusions of the internal carotid artery (ICA) without migraine over a time period of at least 13 years. Initially, the patient had presented with a bilateral ICA occlusion and a cerebral infarct on the right side. While the right ICA remained occluded, a reopening of the left ICA could be detected 3 days after this initial event. In subsequent years, both duplex sonography and magnetic resonance angiography revealed recurrent occlusions of the left ICA, which resolved spontaneously within days. This case and other rare previous reports indicate that recurrent non-migrainous vasospasms of the extracranial carotid artery likely reflect a distinct entity which can cause ischemic strokes.