Carotid artery stenosis (CS) is a major medical problem affecting approximately 10% of the general population 80 years or older and causes stroke in approximately 10% of all ischemic events. In patients with symptomatic, moderate-to-severe CS, carotid endarterectomy (CEA) and carotid angioplasty and stenting (CAS), has been used to lower the risk of stroke. In primary CS, CEA was found to be superior to best medical therapy (BMT) according to 3 large randomized controlled trials (RCT). Following CEA and CAS, restenosis remains an unsolved problem involving a large number of patients as the current treatment recommendations are not as clear as those for primary stenosis. Several studies have evaluated the risk of restenosis, reporting an incidence ranging from 5% to 22% after CEA and an in-stent restenosis (ISR) rate ranging from 2.7% to 33%. Treatment and optimal management of this disease process, however, is a matter of ongoing debate, and, given the dearth of level 1evidence for the management of these conditions, the relevant guidelines lack clarity. Moreover, the incidence rates of stroke and complications in patients with carotid stenosis are derived from studies that did not use contemporary techniques and materials. Rapidly changing guidelines, updated techniques, and materials, and modern medical treatments make actual incidence rates barely comparable to previous ones. For these reasons, RCTs are critical for determining whether these patients should be treated with more aggressive treatments additional to BMT and identifying those patients indicated for surgical or endovascular treatments. This review summarizes the current evidence and controversies concerning the risks, causes, current treatment options, and prognoses in patients with restenosis after CEA or CAS.