BACKGROUND Radiofrequency ablation (RFA) is the criterion standard treatment for patients with atrioventricular nodal reentrant tachycardia (AVNRT). Knowledge about RFA in patients with dextrocardia and situs inversus is limited due to their rare incidence and complexity. The incidence of dextrocardia is reported to be 1 in 12 000 births, with situs inversus occurring in one-third of the cases. The incidence of congenital heart disease is about 5% in these patients. However, data on rhythm and conduction disorders in this group of patients are currently limited, making management more difficult owing to their individual anatomy. CASE REPORT We report the case of an obese 21-year-old man with complex congenital heart disease (CCHD) (situs inversus dextrocardia, pulmonary atresia, single ventricle, common atrium with single atrioventricular valve), asplenia, and multiple cardiac-corrective surgeries (Fontan repair, bidirectional Glenn anastomosis, and Blalock-Taussig shunt) who underwent successful RFA of recurrent supraventricular tachycardia. CONCLUSIONS Supraventricular arrhythmias are common in the setting of CCHD. Although catheter ablation procedures are technically challenging to perform in patients with CCHD, they remain the best therapeutic option for these arrhythmias. To our knowledge, this case is the first to be described in the literature of successful ablation of AVNRT in a patient with situs inversus dextrocardia, pulmonary atresia, a single ventricle, a common atrium with a single atrioventricular valve, and multiple cardiac-corrective surgeries.