INTRODUCTION Situs inversus totalis, dextrocardia with interrupted inferior vena cava and azygos vein continuation concomitant with symptomatic atrial fibrillation requiring ablation. This case was deemed not suitable for percutaneous ablation due to anatomic variations and the lack of case reports in literature. METHODS AND RESULTS We performed bilateral thoracoscopic epicardial ablation and epicardial left atrial appendage exclusion. The direct vision allowed for a complete box lesion set with bipolar radiofrequency device. Patient remained in sinus rhythm at 12-months follow-up. CONCLUSION Surgical thoracoscopic epicardial ablation is safe and effective also in congenital defects. Multidisciplinary expertise can offer minimally invasive ablation treatments.