This manuscript reviews the management of patients with spontaneous dissection of the cervical internal carotid artery (sICAD). Recommendations are not based on controlled-randomized trials, but on case-control and observational, hospital-based studies, and case reports. Vascular risk factors seem to be as prevalent in patients with sICAD as in age-matched, healthy volunteers. Stroke prevention includes the treatment of vascular risk factors and the administration of oral aspirin, which may be as effective as anticoagulation. The few available data indicate that most patients with sICAD causing severe stenosis or occlusion, or an aneurysm can be treated conservatively. Patients with sICAD were not excluded in the intravenous controlled-randomized thrombolysis trials with tissue plasminogen activator, but were excluded in the intraarterial controlled-randomized thrombolysis trials. Taking the few published case series and reports on thrombolysis in patients with sICAD into consideration, intravenous thrombolysis may be beneficial, whereas it remains unclear whether intraarterial thrombolysis is useful.