Introduction: Iliac vein aneurysm is a rare clinical entity. Iliac venous tract is the least commonlocation for venous aneurysms. There are a few cases of common, external and internal iliac veinaneurysms in the literature. However, undiagnosed and ruptured iliac venous aneurysms couldhave hazardous consequences. Herein, we reviewed all literature cases of iliac vein aneurysms.Their potential diagnostic and therapeutic challenges are discussed. Literature Review: Following a systematic search, 50 cases of iliac venous aneurysms wereidentified. We used MEDLINE [1900-March 2018] and EMBASE [until March 2018]. MeSHterms of iliac vein/veins/venous, hypogastric, inferior vena cava and aneurysm/aneurysms wereused. Fifty patients with venous aneurysms located in common, external or internal iliac veinswere found in our systematic search. Seventeen patients were female (35.4%) and 31 patients weremale (64.6%). The age range was 13 to 70 years of age. The aneurysm was located in right side in17 patients (34%). It was located in left side in 29 patients (58%) and it was bilateral in 4 patients(8%). The aneurysm was located in common, external and internal iliac veins in 15 (30%), 31(62%) and 4 (8%) patients respectively. The aneurysm was due to a previous arteriovenousfistula (AVF) in 19 patients (38%) and of them, 16 patients (32%) had a history of AVF resultingfrom a previous trauma. 29 patients (59.2%) underwent open surgical treatment. Five patients(10.2%) underwent endovascular treatment. One patient (2.0%) underwent hybrid treatment.Conservative treatment was used in 14 patients (28.6%). Conclusion: Iliac vein aneurysms are extremely rare. Its diagnosis necessitates precise clinicalsuspicion and the treatment is based on patients’ clinical scenario and radiological features. Bothopen and endovascular techniques could be feasible. Iliac vein aneurysms are more commonin men. Left sided aneurysms are more common. The most common anatomic location isexternal iliac vein. The most common cause of iliac aneurysms is dilatation of vein secondary toa traumatic AVF.