Few studies have been devoted to the natural history of cervical artery dissection (CAD). This review shows that apart from neurological damage due to initial stroke, the prognosis of DAC is good. Indeed, the risk of recurrent DAC or recurrent stroke is very low, under 1% per year in patients without typical connective tissue disease. Patients with connective tissue disease (Ehlers Danlos syndrome or Marfan syndrome) or familial history of arterial dissection have an increased risk of recurrent CAD. However, the number of cases due to these diseases remains very low. The other risk factors for recurrent CAD or stroke remain unknown. The benefit of antithrombotic treatments at the acute phase of CAD or for secondary prevention has been never assessed in a randomized clinical trial.