Loeys-Dietz syndrome (LDS) is a rare life-threatening condition, with autosomal-dominant inheritance, which is caused by heterozygous mutations in the genes encoding transforming growth factor beta receptors 1 and 2. It is characterized by a triad of tortuous arteries and aortic aneurysms, cleft palate or bifid uvula, and hypertelorism. Loeys-Dietz syndrome is a recently-described connective tissue disorder with features similar to those of Marfan syndrome, and the vascular type of Ehlers-Danlos syndrome. In Loeys-Dietz syndrome, the aortic aneurysms are prone to rupture at a smaller size than other aneurysms, putting children with Loeys-Dietz at great risk for dying if the aneurysm is not identified and treated early. Here we report  54-year-old-man who had atrial septal defect,aortic root dilatation and bifid uvula, which  may be a variant of LDS.