BACKGROUND Poorly controlled ventricular rate associated with atrial fibrillation (AF) leads to tachycardia-induced left ventricular dysfunction. Atrioventricular (AV) nodal ablation and cardiac pacing is the standard of care in refractory congestive heart failure (CHF) due to AF with moderate to rapid ventricular response that failed conventional medical therapy. If the patient is not a candidate for AF ablation with pulmonary vein isolation and elimination of AF foci, this is an effective approach, but it does have some challenges when done in a patient with dextrocardia and situs inversus. CASE REPORT Our patient was a 77-year-old woman with dextrocardia and situs inversus, with a history of permanent AF due to severe coronary artery disease (CAD), who suffered from recurrent CHF exacerbations from permanent AF with moderate to rapid ventricular response with underlying hypertensive cardiovascular disease. She was a poor candidate for pulmonary vein isolation because of her permanent AF status and high risk of recurrence. She underwent a technically challenging AV nodal ablation with cardiac pacing due to the complex anatomy, with drastic improvement of symptoms within the next 24 h. CONCLUSIONS AV nodal ablation with cardiac pacing is the standard of care in patients with refractory AF with moderate to rapid ventricular response who have failed medical therapy and are not candidates for pulmonary vein isolation.