Thoracic outlet syndrome (TOS) is a condition caused by compression of the neurovascular structures leading to the arm passing through the thoracic outlet. There are three distinct types of TOS: neurogenic (95%), venous (4%–5%), and arterial (1%). Treatment algorithms depend on the type of TOS. Although statistically the most common type, neurogenic TOS can often be the most difficult to diagnose and treat. We have good follow-up data indicating that appropriately selected patients benefit from surgical intervention. Arterial and venous TOS often present more urgently with arterial or venous thrombosis. The thrombosis is typically recognized expeditiously by thorough history taking and physical examination, augmented by duplex ultrasonography. The restoration of blood flow, be it venous or arterial, often can be accomplished readily by thrombolysis. The key, however, comes in diagnosing the underlying structural component involved in the development of symptoms. To prevent recurrence, patients must undergo first rib resection and anterior scalenectomy, as well as resection of any rudimentary or cervical ribs. In the case of arterial TOS, the subclavian artery often requires reconstruction as well. Regardless of the type of TOS encountered, proper treatment requires a multidisciplinary approach.