Spontaneous cervicocephalic arterial dissection (SCAD) has been recognized as an uncommon cause of cerebral stroke. Although it has been viewed as an important cause of stroke, especially in juvenile cases, its natural course and pathophysiology have yet to be fully clarified, and no treatment criteria have been established. Recent studies have suggested that clinical features of SCAD in Japan are different from those in European countries. Herein, we reviewed the current status of the management of SCAD in Japan, and clarified its clinical characteristics to establish an appropriate treatment.