ABSTRACT
Obstruction of the inferior vena cava may presented with unusual and varied pathologic condition and be due to thrombus, extension of tumor, extrinsic compression, or intrinsic caval disease. Thrombus is major cause of obstruction of the inferior vena cava and congenital absence of the inferior vena cava with azygos continuation is extremely rare. In congenital absence of the inferior vena cava (Infrahepatic interruption of inferior vena cava with azygos continuation), infrahepatic inferior vena cava is obliterated but infrarenal portion of the inferior vena cava is patent. In this anormaly, blood from the lower extremities and kidney drained into azygous vein and hemiazygous vein via ascending lumbar vein which eventually into the superior vena cava. These anormaly is seen in 0.6% of patients with congenital heart disease, particularly in cases of polysplenia. We experienced one case of congenital absence of the infrahepatic inferior vena cava, which involved infrarenal portion and the common iliac vein with azygos continuation in 63-year-old male. He was complained of lower abdominal pain, constipation. We report this case with reviewing literatures.