A 70-year-old male is described who suffered from daily episodes of supraventricular tachycardia that was refractory to oral medical treatment since adolescence. Electrocardiographical and electrophysiological evaluation confirmed atrioventricular nodal re-entrant tachycardia (AVNRT). This arrhythmia commonly occurs in patients without concomitant heart disease. However, in this patient a variant form of the scimitar syndrome was found by coincidence at the age of 56 years. This report describes (1) the variant form of the scimitar syndrome, (2) the coexistence of AVNRT in scimitar syndrome, and (3) the feasibility of radiofrequency catheter ablation of AVNRT in the presence of this congenital anomaly.