Marfan syndrome is an autosomal dominant systemic disorder of the connective tissue. Children affected by this syndrome carry a mutation in one of the two copies of the gene encodingthe protein fibrillin-1. It affects most organs and tissues, especially the skeleton, lungs, eyes, heart, and the large blood vessel that distributes blood from the heart to the rest of the body. A case report of Marfan syndrome has been reported which was diagnosed in the dental set-up.