Intracranial atherosclerotic disease (ICAD) is a major cause of ischemic stroke, with higher occurrence rates in Asian, black and Hispanic individuals than in white individuals. Important risk factors for ICAD include age, hypertension, diabetes mellitus and metabolic syndrome. ICAD patients have a 10–20% annual risk of stroke, with greater risk in patients with high-grade stenosis (70–99%). Given the high risk of stroke recurrence in symptomatic ICAD patients, effective secondary prevention strategies are needed. Currently, there is no strong evidence supporting the use of anticoagulants in ICAD patients; antiplatelet drugs are still the preferred medication. Recently, angioplasty and stenting for ICAD has emerged as an alternative approach to medical treatment, owing to high technical success rates and low risk of periprocedural complications. However, the rate of in-stent restenosis is moderate, and the long-term benefit of endovascular treatment is not well established. High-quality randomized trials comparing angioplasty/stenting with the best medical therapy in ICAD patients are anticipated.