Congenital anomalies of the inferior vena cava is an extremely rare vascular anomaly with controversial pathogenesis. Anomalies of the inferior vena cava (IVC) are present in 0.3% to 0.5% of otherwise healthy individual and in 0.6% to 2% of patients with other cardiovascular defects. The phenomenon of absence of the IVC has been described in a variety of ways such as absence, agenesis, anomalous, and interruption of a particular segment ([infra] hepatic, prerenal, renal, or infrarenal) of the IVC. We describe a 42-year-old man with chronic venous insufficiency, without DVT, caused by congenital absence of renal segment of the IVC.