An 8 1/2-month-old girl with biliary atresia and polysplenia syndrome having multiple vascular anomalies without cardiac anomalies is reported. Interruption of the inferior vena cava with azygous continuation, which is a common anomaly, was seen in conjunction with origin of the common hepatic artery from the superior mesenteric artery and with a circumaortic renal vein. The case has particular importance in that no hepatic artery or renal vein variations have been described with biliary atresia and polysplenia syndrome in the literature thus far to our knowledge. The anomalies were shown using different radiological examinations including computed tomography, echocardiography, angiography, venography and magnetic resonance imaging.