Spontaneous dissection of the extracranial internal carotid artery is uncommon and has been first described in 1959. Since then, 250 cases have been published. The authors report on 5 recent cases and on that occasion, they review the literature and discuss the different therapeutic possibilities. This pathology can be envisaged when a middle-aged adult, free of any previous pathology or trauma, presents with latero-cervical pain followed by cerebral ischemic stroke. The angiographic findings are of three types: regular or irregular extensive stenosis, pseudo-aneurysmal dilatation followed by luminal stenosis, funnel-like tapering of the vessel. The spontaneous course of such a dissection most often (80%) leads to an almost complete resolution of clinical and angiographic signs. Treatment, therefore, should essentially be based on anticoagulant and antiaggregant therapy, so as to prevent ischemic attacks or carotid thrombosis. Surgical indications should remain exceptional.