This report demonstrates an angiography of azygos continuation of inferior vena cava with polysplenia. In this patient computed tomography with contrast enhancement, magnetic resonance angiography of the thorax with TOF technique and angiography of IVC by seldinger technique with entering right main femoral vein was performed. It was exhibited azygos continuation of IVC, polysplenia and hepatic veins which connected to the right atrium. Congenital malformations of IVC are rare. Patients are usually asymptomatic and this developmental anomaly is detected incidentally during abdominal surgery or radiologic evaluation. The recognition of this congenital venous anomaly is important for the cardiopulmonary bypass or palliative systemic venous–pulmonary artery shunt surgery, IVC filter placement and cardiologist especially for conditions such as venous thromboembolism.