Background: Trans-catheter ablation of drug refractory atrial fibrillation (AF) is an effective and safe treatment to avoid arrhythmia recurrences. However, some patients may present anatomic variant making impossible an inferior venous approach. We report a case of pulmonary veins isolation (PVI) with cryoballoon technology via right internal jugular vein (RIJV) access. Methods: After the first trans catheter PVI attempt was failed due to inferior vena cava (IVC) hypoplasia, cryoballoon ablation with a 28-mm cryoballoon (AFAPRO - Medtronic CryoCath LP, Quebec, Canada) via superior venous access was performed. A quadripolar electrode was inserted in coronary sinus from left basilic vein. The right internal jugular vein was accessed with Seldinger technique and left atrial (LA) catheterization was performed via single trans-septal puncture (TP) under trans-esophageal echocardiographic (TOE) and fluoroscopy guidance using an SL2 (St. Jude Medical) trans-septal sheath with a BRK2 (St. Jude Medical) needle. All pulmonary veins (PVs) were engaged. Cryothermal energy was delivered after checking the correct occlusion and PVI was successfully obtained for all PVs. The procedure lasted 210 min, fluoroscopy time was 55 min. During the 16-month follow-up no sustained atrial arrhythmia recurrence was detected. Conclusion: PVI with cryoballoon technology in patients with hypoplasia of IVC can be safely and successfully performed using right internal jugular access.