IntroductionCatheter ablation of atrial fibrillation (AF) has emerged as the most effective therapy. However, rare anatomical abnormalities such as situs inversus totalis, dextrocardia, or interrupted inferior vena cava can make ablation challenging.Methods and ResultsWe report a case of a 55‐year‐old woman with situs inversus totalis, dextrocardia, surgical atrial septal defect repair, left‐sided dual chamber pacemaker in place, and symptomatic recurrent persistent AF who underwent successful pulmonary vein and posterior wall isolation by the superior access from the left internal jugular vein.ConclusionsIt is a feasible and safe approach with support of transesophageal echocardiography and multiple emerging technologies.