Approximately 10% of all cases of deep vein thrombosis (DVT) occur in the upper extremities. The most common secondary cause of upper-extremity DVT (UEDVT) is the presence of a venous catheter. Primary UEDVT is far less common and usually occurs in patients with anatomic abnormalities of the costoclavicular space causing compression of the subclavian vein, called venous thoracic outlet syndrome (VTOS). Subsequently, movement of the arm results in repetitive microtrauma to the vein and its surrounding structures causing apparent ‘spontaneous’ thrombosis, or Paget-Schrötter syndrome. Treatment of UEDVT aims at elimination of the thrombus, thereby relieving acute symptoms, and preventing recurrence. Initial management for all UEDVT patients consists of anticoagulant therapy. In patients with Paget-Schrötter syndrome the underlying VTOS necessitates a more aggressive management strategy. Several therapeutic options exist, including catheter-directed thrombolysis, surgical decompression through first rib resection, and percutaneous transluminal angioplasty of the vein. However, several controversies exist regarding their indication and timing.