Ebstein's anomaly is defined as the significant apical displacement of the tricuspid valve causing tricuspid regurgitation. Although a variety of concomitant lesions have been previously described, we herein introduce an unusual presentation. Our patient was an 86-year-old man with a primary presentation of typical chest pain in the setting of recently diagnosed coronary artery disease with concomitant Ebstein's anomaly. We found mild-to-moderate tricuspid regurgitation, bicuspid aortic valve, persistent left superior vena cava, and patent foramen ovale. The patient had suffered from chest discomfort on exertion for 2 months with good functional capacity prior to diagnosis. Coronary angiography revealed two-vessel disease. The patient refused surgery. He was treated with medical anti-ischemic therapy. He had good exercise tolerance with relief of chest pain at the latest follow-up. The features demonstrated in this case report suggest that there may be several adult survivors of complex congenital heart diseases requiring individualized surgical treatment plans.