The Journal of Tehran University Heart Center Here we introduce a family (Figure1), eleven members of whom had MFS. The family lived in the south-eastern Iranian city of Birjand, and we have followed it up all for ten years now. Cases Report In 1998, a 17-year-old male (second son of this family) was referred to an ophthalmologist for visual disturbances. The ophthalmologic examination revealed lens displacement, iridodenosis, and high intraocular pressure. The patient’s tall stature and arachnodactily (Figures 2 & 3) arouse the suspicion of MFS and he was referred to a cardiologist for further investigation. Echocardiography further established the diagnosis of MFS by demonstrating aortic root dilation and mitral valve prolapse (Figure 4). Figure 3. Positive thumb sign Mohammad Hossein Davari et al. Figure 2. Positive wrist sign and arachnodactily Figure 4. Parasternal view of echocardiography, showing aortic root dilatation AO, Aorta; LA, Left atrium; LV, Left ventricle; RA, Right atrium; RV, Right ventricle Figure 5. Pedigree of the family 38