Among the large variety of cerebrovascular syndromes, carotid artery dissections remain often under-recognized. Many patients present with minor or transient symptoms, some with local pain only, and some are asymptomatic. A useful rule is that the coincidence of any local pain in neck or face with an ipsilateral Horner's syndrome preceding transient or persistent retinal or cerebral ischemic symptoms should alert physicians--notably those treating emergencies--to rule out an underlying dissection of the ipsilateral carotid artery. In most cases the extracranial part of the carotid artery is affected; therefore, this review will focus on this main subgroup of patients. We review the pathological anatomy, as well as possible underlying causes, clinical features, diagnostic tools, differential diagnosis, treatment options, and outcome data.