Congenital anomalies of the inferior vena cava (IVC) and its tributaries have become more commonly recognized in asymptomatic patients. The embryogenesis of the IVC is a complex process involving the formation of several anastomoses between three paired embryonic veins. The result is numerous variations in the basic venous plan of the abdomen and pelvis. A left IVC typically ends at the left renal vein, which crosses anterior to the aorta to form a normal right-sided prerenal IVC. In double IVC, the left IVC typically ends at the left renal vein, which crosses anterior to the aorta to join the right IVC. In azygos continuation of the IVC, the prerenal IVC passes posterior to the diaphragmatic crura to enter the thorax as the azygos vein. In circumaortic left renal vein, one left renal vein crosses anterior to the aorta and another crosses posterior to the aorta. In retroaortic left renal vein, the left renal vein passes posterior to the aorta. In circumcaval ureter, the proximal ureter courses posterior to the IVC. Other anomalies include absence of the infrarenal IVC or the entire IVC. These anomalies can have significant clinical implications. Awareness of these anomalies is necessary to avoid diagnostic pitfalls.