No randomized and controlled study has evaluated acute stroke therapy and antithrombotic agents for stroke prevention in patients with spontaneous cervical artery dissection (CAD). CAD was not a contraindication for including cases with acute ischemic stroke in trials evaluating systemic fibrinolysis with recombinant tissue plasminogen activator (rt-PA). Small case series have reported successful systemic and local intra-arterial thrombolysis without clinical signs of rupture of the dissected vessel. Thus, thrombolysis seems to be a therapeutic option in acute CAD causing ischemic stroke, although it remains unclear whether rt-PA increases the obstruction of the dissected vessel by enlarging the wall hematoma or diminishes the obstruction by enhancing the recanalization of the thrombus adhering to the dissection. Meta-analyses have shown no benefit of anticoagulation compared to aspirin in stroke prevention of patients with CAD. It is also unclear, whether long-term antithrombotic therapy is necessary. Many centers and ours maintain the antithrombotic therapy for 3 to 6 months. Dissections of the internal carotid artery (ICAD) have a benign long-term prognosis with low stroke rates that are not related to the persistence of severe carotid stenosis or occlusion. These results suggest that surgical or endovascular therapy of ICA stenosis or occlusion related to ICAD should only be taken into consideration in the very rare patients with stroke recurrence in spite of an optimal medical treatment. Cervical aneurysms caused by CAD have an excellent long-term outcome with a low stroke risk, and no vessel rupture has been reported. Thus, surgical or endovascular therapy should be restricted to the very rare cases developing ischemic symptoms in the vascular territory supplied by the dissected aneurysm in spite of antithrombotic therapy.