Arterial thoracic outlet syndrome (TOS) is the least common form of TOS in adults. It is an entity that is associated with bony anomalies resulting in chronic subclavian artery compression. Most patients with arterial TOS are young adults presenting either with limb-threatening upper extremity ischemia or chronic symptoms suggestive of arterial insufficiency involving the extremity. Initial diagnostic evaluation involves chest radiography, which may reveal cervical or anomalous first rib. Catheter-based arteriography has a diagnostic as well as therapeutic role. Magnetic resonance angiography and computed tomographic angiography, which are readily available, can be used in surgical planning. Treatment involves revascularization of the extremity, subsequent first rib resection, and possible reconstruction of the subclavian artery. This review contains 4 figures, 1 table and 45 references Key Words: arterial complication, brachial thromboembolectomy, cervical rib, costoclavicular space, first rib resection, pectoralis minor space, scalene triangle, subclavian artery stenosis, thoracic outlet syndrome