Marfan syndrome is an uncommon inheritable connective tissue disease which affects the cardiovascular system. This paper presents two cases of Marfan Syndrome with predominant aortic root disease that were seen at the Cardiology Clinic of University of Nigeria Teaching Hospital, Enugu, Nigeria. Their biography, clinical features and echocardiography parameters were captured using structured questionnaire. Both were young males in their 4th decade of life, and had advanced aortic root diseases which were complicated by left ventricular failure in both, while one of them had aortic dissection and ischemic stroke. Young adult Nigerians with Marfan syndrome presented with advanced aortic root diseases, heart failure, aortic dissection and stroke.