Upper extremity deep vein thrombosis (UEDVT) is associated with significant morbidity and mortality. The susceptible populations and risk factors for UEDVT are well-known. The presenting symptoms can be subtle, and therefore objective testing is necessary for diagnosis. The optimal diagnostic strategy has not been determined, and more than one test may be required to exclude the diagnosis. Proper treatment reduces the occurrence of complications, and treatment should include long-term anticoagulation if the patient has no contraindications. This article discusses the risk factors, pathogenesis, diagnosis, complications, and management of UEDVT.