Stenosis of the vertebral artery (VA) in either its extra- or intracranial portions is an important cause of posterior circulation stroke. Diagnosis of VA stenosis by noninvasive imaging techniques is improving and new endovascular and medical treatments are now available. However, the natural history of VA stenotic lesions is not known and its optimum management is unclear. Symptomatic VA stenosis should be initially treated with established antiplatelet agents. There is no proven indication for anticoagulation in cases of VA stenosis. Case series have shown that angioplasty and stenting for proximal extracranial VA stenosis have a low perioperative complication rate and are effective in restoring luminal diameter. Evidence from randomized trials on its long-term efficacy versus medical therapy is not available. Regarding current evidence where symptoms are refractory to antiplatelet treatment, or where recurrent stroke risk is considered increased due to either an incomplete circle of Willis or an anomalous VA circulation, it can be considered in centers with experience of the procedure. Randomized trials comparing stenting with medical therapy are required. Surgery may be a viable alternative to angioplasty and stenting, but only in a few specialist centers. Evidence from randomized controlled trials of medical and other interventions for VA stenosis are required.