The duplication of the inferior vena cava (IVC) is a rare congenital anomaly, which also has some variations regarding the complex embryological development of the IVC. In the typical form, infrarenal IVC segments are duplicated and the left IVC joins the left renal vein, which crosses anterior to the aorta in the normal fashion to join the right IVC. In variant forms, the interruption of the intrahepatic segment of the IVC, azygos or hemiazygos continuation, or retroaortic course of the renal vein may be seen. An intrahepatic venous shunt accompanying a double IVC variant is an extremely rare anomaly. We report a case of 40-year-old female patient with double IVC, hemiazygos continuation, intrahepatic IVC interruption, and a transhepatic venous shunt.
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