The aim of this article is to review the published English literature on aetiology, pathology, clinical presentation, diagnostic methods and treatment of renal vein thrombosis.We searched the published literature from Medline & Pubmed using keywords renal vein thrombosis, anti-phospholipid syndrome and nephrotic syndrome. Data was extracted from individual case reports, case series, articles on pathology, diagnostic tests, treatment modalities, and previous reviews. Case reports which did not add any new information were excluded.We selected 60 references based on the above criteria. Renal vein thrombosis is relatively rare. CT angiography is considered the investigation of choice. Alternatives include MR angiography or renal venography in highly selected patients. As the condition is relatively uncommon, consensus on the best form of therapy for this condition has been slow to evolve. The trend in management has shifted to non-surgical therapies particularly systemic anticoagulation except in highly selected group of patients.