Post-thrombotic syndrome is a chronic, potentially debilitating complication of deep vein thrombosis (DVT) of the lower extremity. Comparatively little is known about post-thrombotic syndrome after upper extremity DVT (UEDVT). To perform a systematic review of clinical studies that have examined the incidence, clinical features, risk factors and management of post-thrombotic syndrome after UEDVT. Using combinations of keywords venous thrombosis, postphlebitic syndrome, thrombophlebitis, arm swelling, post-thrombotic syndrome, UEDVT, Paget-Schroetter syndrome, thoracic outlet syndrome, axillary vein, subclavian vein, and central venous catheter, the MEDLINE database was searched for English language articles published between January 1967 and December 2004. Retrieval and review of articles were restricted to clinical studies in humans that described long-term outcomes after objectively confirmed UEDVT. Seven studies were reviewed. The frequency of PTS after UEDVT ranges from 7-46% (weighted mean 15%). Residual thrombosis and axillosubclavian vein thrombosis appear to be associated with an increased risk of PTS, whereas catheter-associated UEDVT may be associated with a decreased risk. There is currently no validated, standardized scale to assess upper extremity PTS, and little consensus regarding the optimal management of this condition. Quality of life is impaired in patients with upper extremity PTS, especially after DVT of the dominant arm. PTS is a frequent complication of UEDVT, yet little is known regarding risk factors and optimal management. A standardized means of diagnosis would help to establish better management protocols. The impact of upper extremity PTS on quality of life should be further quantified.