The incidence of upper extremity deep vein thrombosis (UEDVT) is constantly increasing due to frequent insertion of intravenous devices for various indications. Secondary UEDVT represent more than two thirds of cases. Primary UEDVT generally include UEDVT occurring without any triggering factor and UEDVT following intense or repeated efforts or in association with the thoracic outlet syndrome, with a causative link that may be sometimes very difficult to ascertain. As opposed to lower extremity DVT, data remain very scarce and of limited quality in this setting. Diagnosis is mainly based on clinical probability assessment associated with D-dimer measurement and compression ultrasound as first-line imaging. Treatment still consists of therapeutic anticoagulation in the vast majority of cases.