Aneurysms of the inferior vena cava (IVC) are extremely rare. To the best of our knowledge, only 29 cases of IVC aneurysms are published in literature. We present a new case of surgically treated symptomatic saccular aneurysm of the infrarenal IVC and review previously published cases. Following resection of the aneurysm and the thrombosed infrarenal IVC, the patient fully recovered. Thrombosed IVC aneurysm may mimic a retroperitoneal tumour. In some cases, CT and MRI findings may be equivocal. Surgical treatment is indicated in all symptomatic and low-risk asymptomatic cases.