Congenital anomalies of the inferior vena cava and its tributaries are quite uncommon; however, since the development of cross-sectional imaging, they have been described more frequently. Most of them are usually asymptomatic, although they can have clinical implications. We report a case of a rare anomaly of the inferior vena cava in a 54-year-old man admitted to our hospital for complicated native aortic valve endocarditis, requiring urgent surgery, and making necessary an alternative venous cannulation for establishing cardiopulmonary bypass circulation.