The iliac veins are the least frequent location for venous aneurysms, with only a few cases described globally. The etiology and clinical presentation of this extremely rare entity is diverse and unclear and no treatment consensus has been reached yet. Our purpose is to present an interesting iliac vein aneurysm (IVA) case that we treated in our department, with a brief review of the literature. We report a case of a 74-year-old male patient with a giant, 55 mm in diameter, asymptomatic, right common IVA, with concurrent aplasia of the left common iliac vein and an extensive network of venous collaterals. The patient was treated, under general anesthesia, with total endovascular iliocaval reconstruction through implantation of a 32 × 100 mm thoracic aortic tubular Ankura stent graft. The computed tomography venography at first-month follow-up showed the complete exclusion of the IVA, without any endoleak and the patient remains up to date free of symptoms and thromboembolic events. Twelve cases of endovascular treatment of IVA have been reported so far, and our case is the first with implantation of a thoracic aortic stent graft. Our results suggest that this technique is safe, effective, and may be considered for appropriately selected patients. This is the first case with total endovascular repair of an iliac vein aneurysm with contralateral iliac vein aplasia through endovenous implantation of a thoracic aortic stent-graft. Our results suggest that this technique is safe and effective and thus, may be considered for appropriately selected cases.