Tehran University of Medical Sciences The Research in Heart Yield and Translational Medicine (RHYTHM) 3115-7270 12 1 2017 03 05 39 41 EN Bahieh Moradi Day General Hospital, Tehran, Iran. Farideh Roshanali Day General Hospital, Tehran, Iran. 2017 02 11 2017 02 11 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. https://rhythm.tums.ac.ir/index.php/jthc/article/view/678 https://rhythm.tums.ac.ir/index.php/jthc/article/download/678/524