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The differential diagnosis of BVH with other congenital and familial diseases in which increased ventricle wall thickness is a prominent clinical feature is fundamental due to its therapeutic and prognostic value, mainly during infancy. We describe a 2\u2010month\u2010old infant presenting BVH. Using exome sequencing, we identified a novel de novo 3\u2010bp deletion in the <jats:italic>RAF1<\/jats:italic> gene that is located in the binding active site for the 14\u20103\u20103 peptide. Based on docking calculations, we demonstrate that this novel mutation impairs protein\/target binding, thus constitutively activating Ras signaling, which is a dysregulation associated with Noonan syndrome. 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