Marfan syndrome (MFS) is an inherited connective tissue disorder. The severity of Marfan syndrome varies from one individual to another and it typically progresses over time. A tall, slender stature is characteristic of Marfan syndrome. It affects the skeleton, eyes, heart, nervous system, skin and respiratory system. It is caused by a defect or mutation in the gene that determines the structure of fibrillin-1, a protein that is an important part of connective tissue. Cardiovascular complications are the most common cause of patient death. Aortic dissection and congestive heart failure due to aortic and mitral valvular anomalies accounted for over 90% of the known causes of death. Here, we reporta case of this rare genetic connective tissue disorder. Keywords: - Marfan syndrome, Ectopia lentis, Skeletal system,Aortic regurgitation.