Acute occlusion of the internal carotid artery is the underlying etiology in 4 to 15% of all ischemic strokes. The clinical presentation varies considerably ranging from asymptomatic occlusion to severe ischemic strokes. Substantial differences in the acute management of acute symptomatic internal carotid artery occlusions (ICAO) exists between centers. Thusly, we comprised a narrative review of the natural course of acute ICAO and of available treatment options [i.v. thrombolysis, endovascular thrombectomy and stenting, bypass between the superficial temporal and the middle cerebral arteries (MCA) and carotid endarterectomy (CEA)]. We found that very few randomized treatment trials have been performed in patients acute symptomatic ICAO. Most evidence stems from case series and observational studies. Especially in older studies the intracranial vessel status has rarely been considered. After revision of these studies we concluded that the mainstay of the acute management of acute symptomatic ICAO is i.v. thrombolysis when applied within the label and in combination with mechanical thrombectomy in case of intracranial large vessel occlusion. In cases without intracranial large vessel occlusion mechanical thrombectomy of acute ICAO is associated with a risk of distal embolization. More research on prognostic parameters is needed to better characterize the risk of decompensation of collateral flow and to better define the time-window of intervention. When mechanical thrombectomy fails or is not available, surgical approaches are an alternative in selected patients.