Three VUS variants in FBN1 (p.Tyr20Cys, p.Ala1152Val, and p.Glu2019Lys) were previously reported in patients with Marfan syndrome, without sufficient evidence to be classified as likely pathogenic (Arbustini et al. 2005; Hung et al. 2009; Sheikhzadeh et al. 2012). This evidence concerns the gene FBN1 and Marfan syndrome.