Failure of normal embryogenesis may result in various anomalies of the inferior vena cava that can bereli-ably detected by computed tomography. Agenesis of the intrahepatic segment of the inferior vena cava withazygos continuation is a rare anomaly due to a complex developmental process of the inferior vena cava. Theauthors report a case in which this anomaly was diagnosed on computed tomography and confirmed by inferi-or venacavography.