Upper extremities deep venous thrombosis (UEDVT) is a rare condition. According to the literature, approximately 4-10% of all cases of venous thrombosis may involve the subclavian, axillary or brachial veins. In the last few decades, the incidence of UEDVT has increased because of more frequent use of central venous catheters (CVCs) and cardiac pacemaker implantation. In addition, another common risk factor for UEDVT is cancer. UEDVT is classified as primary, approximately one-third of cases, which refers either to effort thrombosis or idiopathic UEDVT, or secondary, due to the presence of overt predisposing causes. The onset of UEDVT is usually characterized by arm swelling and pain, but may also be completely asymptomatic especially in patients with a long-term presence of a CVC. Ultrasonography represents a simple and accurate diagnostic tool to demonstrate the problem. UEDVT has major clinical consequences including pulmonary embolism, recurrences, post-thrombotic syndrome, and death. The role of thromboprophylaxis for those patients with a long-term CVC is still controversial. Unfractionated or low molecular weight heparin, followed by an oral anticoagulant are the most common treatments, with strategy of management similar to that of deep vein thrombosis of the leg. Thrombolysis/thrombectomy and surgical decompression are often successful, but less frequently used. Randomized controlled trials are warranted to clarify the optimal management of UEDVT, and to identify patients at the highest risk of recurrence who might benefit from long-term anticoagulation.