The optimal surgical management of subclavian vein effort thrombosis remains a dilemma because outcomes after different treatment strategies are only on the basis of small retrospective series. SVT treatment should be on the basis of the cause of thrombosis. Primary effort thrombosis or Paget-Schroetter syndrome frequently necessitates a surgical approach. The type of surgery has to be individualized after careful diagnostic evaluation. We report a congenital clavicular exostosis that had not been identified with chest radiograph and computed tomographic scan that caused SVT in a young woman. This unusual cause of Paget-Schroetter syndrome was treated with a unique approach.