Interrupted inferior vena cava (IVC) with azygous continuation to the superior vena cava (SVC) is a relatively common systemic venous anomaly. This anomaly can occasionally complicate transcatheter intervention by rendering more difficult the usual direct route to the systemic venous atrium afforded by femoral venous access. We report our experience with successful transcatheter closure of a large residual atrial septal defect (ASD) using the femoral venous route in a 3-year-old patient with heterotaxy syndrome of left isomerism type, dextrocardia, partial atrioventricular canal defect, and interrupted IVC with azygous continuation to the SVC.