RESUMEN
Ebstein's anomaly is a relatively rare congenital heart disorder characterised by downward displacement of septal and posterior leaflets of the tricuspid valve into the right ventricle cavity. The usual presenting symptoms are cyanosis, right-sided heart failure and arrhythmia. Progressive heart failure or tachyarrhythmia may worsen cyanosis. The acute coronary syndrome is rarely reported in Ebstein's anomaly. We report a patient of undiagnosed Ebstein's anomaly who was apparently asymptomatic but presented with the acute coronary syndrome. This case report deals with a rare combination of congenital heart disease (Ebstein's anomaly) and coronary artery disease. Ebstein's anomaly (EA) has a prevalence of 1% of all congenital heart diseases, and little evidence is reported in the literature where EA along with coronary artery disease (CAD) exists in individuals less than 45 years old. Therefore, this case report brings attention to the rarity of those pathologies, which individually are already considered rare. And in this case, the association turns this diagnosis exceptional and highlights the complexity of the treatment.