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Spontaneous recanalization of a chronic total occlusion of the extra-cranial internal carotid artery is an under-reported clinical entity. This paper reviews the different etiologies of internal carotid artery occlusion, its natural course, as well as the significance and our recommendations for the management of spontaneous internal carotid artery recanalization. A review of literature on etiology, diagnosis, and treatment of internal carotid artery occlusion and recanalization was conducted. PubMed database was searched using the term