Upper extremity deep vein thrombosis (UEDVT) is responsible for 4 to 10% of all deep vein thrombosis (DVT). Untreated UEDVT can lead to significant disability secondary to the postthrombotic syndrome. To date, there are no randomized trials specifically comparing different therapeutic strategies. Ultimately, optimal management of UEDVT depends on the underlying etiology, patient symptoms, and degree of thrombosis, with supporting evidence primarily extrapolated from lower extremity DVT data. This article will review the classification, presentation, and diagnosis of both primary and secondary UEDVT. In addition, it will discuss updates in clinical guidelines, anticoagulation, endovascular and surgical treatment strategies.