A 59-year-old male with paroxysmal atrial fibrillation refractory to class IC and class III antiarrhythmic drugs was admitted to our hospital for radiofrequency catheter ablation of atrial fibrillation. During preoperative examination situs inversus totalis was revealed. Pulmonary vein (PV) isolation was successfully performed with atrial fibrillation termination and elimination of all PV potentials. The procedure was performed without any complications. Our report shows that PV isolation for treatment of drug-refractory atrial fibrillation can be safely performed in patients with dextrocardia and situs inversus totalis.