In the present report we describe a patient (a 36-year-old woman with 15 year history of supraventricular tachyarrhythmias) with congenital absence of inferior vena cava (IVC) revealed during radiofrequency (RF) catheter ablation procedure for right postero-septal Wolff-Parkinson-White syndrome (WPW). For the absence of IVC, the ablation procedure was more difficult, because we had to perform the ablation with the catheters (the ablator catheter and the coronary sinus catheter) introduced both through the superior vena cava. The application of RF energy (35 Watt for 60 seconds) at successful site abolished accessory pathway conduction. The following day was performed the venous angiography, showing the absence of the IVC and a venous return via paravertebral venous plexus to the azygous vein and superior vena cava into the right atrium. Computer tomography confirmed the absence of the IVC with azygous continuation. The drainage via the azygous system modified the radiological image on chest roentgenogram of the right mediastinal silhouette. During cardiogenesis fusion of the IVC and organisation of the heart occur between the 33rd to 40th embryonic days. It is therefore possible that some unknown teratogenic mechanism at this critical period might have caused, in the patient, both the developmental arrest of IVC and failure of regression of atrio-ventricular anatomical and electrical continuity in the right postero-septal region.