The intention of this article is to report a case of a 26 years old man presenting a rare combination of congenital heart disease (Ebstein anomaly) and coronary artery disease. EA (Ebstein anomaly) has a prevalence of 1% of all congenital heart diseases, as CAD (coronary artery disease) has a little evidence in literature's registries when occurred in individuals with 40 years old or less. Therefore, this case report rises attention to the rarity of those patologies, which individually are already considerably rare and, in this case, it comes associated what turns this diagnosis excepcional, highlighting the complexity of the treatment.