Atherosclerosis of the brachiocephalic vessels is uncommon in comparison to the prevalence of disease in the extracranial carotid arteries. Prior to 1980, occlusive disease of this vascular bed had been treated primarily with surgical intervention, which carried a significant rate of morbidity and mortality. In the last 30 years, however, minimally invasive endovascular techniques have become an important tool in the management of symptomatic patients. Angioplasty and stenting have been reported as a viable therapeutic option, producing good technical success and durability. Here, we examine the natural history of subclavian artery occlusive disease, along with the diagnostic evaluation and various treatment modalities currently available, with special attention paid to the evolution of the role of endovascular management.