Tehran University of Medical Sciences The Research in Heart Yield and Translational Medicine (RHYTHM) 3115-7270 6 1 2011 03 15 45 47 Mohammad Yusef Aarabi Moghadam Shaheed Rajaei Cardiovascular Medical and Research Center, Tehran University of Medical Sciences, Tehran, Iran Maryam Moradian Shaheed Rajaei Cardiovascular Medical and Research Center, Tehran University of Medical Sciences, Tehran, Iran Nader Givtaj Shaheed Rajaei Cardiovascular Medical and Research Center, Tehran University of Medical Sciences, Tehran, Iran Kambiz Mozaffari Shaheed Rajaei Cardiovascular Medical and Research Center, Tehran University of Medical Sciences, Tehran, Iran 2015 10 03 Primary cardiac tumors are quite rare, especially in the pediatric age group, and their atypical presentations often prevent a timely diagnosis. Most primary cardiac tumors in the pediatric age group are benign. Fibromas are generally reported as the second most common primary cardiac tumors in the pediatric age group. These neoplasms are often intramural and involve the left ventricular free wall or the interventricular septum. Although benign, fibromas may become life-threatening by causing arrhythmias or obstruction to the blood flow. A case of supravalvular intraluminal ascending aorta fibroma in a 23-month-old girl, presenting with syncope, is described here; the location is rare and the presentation atypical for this type of tumor. Transesophageal echocardiography helped us to evaluate the anatomic details of the tumor and plan surgery. https://rhythm.tums.ac.ir/index.php/jthc/article/view/200 https://rhythm.tums.ac.ir/index.php/jthc/article/download/200/198