Primary cardiac fibroma of the left ventricular myocardium is a rare tumor of the heart which is usually located within the anterior wall and /or septum of the left ventricle and is the second most common cardiac tumor in infant and children. Although the tumor is benign histologically, it may cause severe cardiac dysfunction and sudden death. A 30-day-old neonate with a huge intramural fibroma involving the posterolateral wall of the left ventricle underwent it`s partial resection under extracorporeal circulation on April 1992.