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Ebstein′s anomaly is a rare congenital heart disease involving the right side of the heart with typical malformations of the tricuspid valve and the right ventricle. Associated left heart anomalies, particularly aortic valve disease, are extremely rare. We report here an unusual case of Ebstein′s anomaly of the tricuspid valve and severe aortic stenosis who presented to us with recurrent syncopal episodes. The patient needed to undergo electrophysiological evaluation before aortic valve replacement to rule out arrhythmic causes of syncope.