Objective: The objective of this article is to provide up-to-date information about aetiology, pathogenesis, diagnostic modalities and treatment of upper limb deep vein thrombosis (ULDVT). Methods: Generic terms including ULDVT, axillary-subclavian DVT, and complications of central venous catheters were searched on electronic database. We analysed original studies, review articles and evaluation studies published over the last 25 years. Results: Forty-seven studies on ULDVT encompassing 2,557 patients were evaluated. The incidence of ULDVT was quoted 1–4% of the total DVT. Primary ULDVT (20% of the total) was due to activity-related venous trauma. Secondary ULDVT (80% of the total) was due to central venous catheters and malignancy. Duplex ultrasound (sensitivity 78–100% and specificity 82–100%), contrast venography (gold standard) and magnetic resonance venography were the diagnostic tools used. Pulmonary embolism (2–35%) and post-thrombotic syndrome (7–46%) were the main sequelae. Anticoagulation was the universal intervention, giving 79% symptom relief (13.2% rethrombosis rate). Thrombolysis and/or percutaneous thrombectomy were used in 38% of cases for the management of ULDVT, giving 83% symptom relief (90% recanalization rate and 9% rethrombosis rate). Surgical decompression, venous angioplasty and superior vena cava filters were the main adjunctive interventions. Conclusion: ULDVT, although rare, is associated with considerable morbidity and mortality (29–40%) due to potential risks of pulmonary embolism, post-thrombotic syndrome and loss of vascular access. Simple anticoagulation is suitable for the majority of patients. Thrombolysis/thrombectomy is often successful but less frequently used. Surgical decompression, venous angioplasty and superior vena cava filters have some role in recurrent cases. An optimal management protocol can be established using a multimodality approach.