Ogden syndrome is an extremely rare X-linked recessive genetic disorder caused by heterozygous mutations in the NAA10 gene, which encodes N-α-acetyltransferase 10, a protein involved in protein biosynthesis.[1,2] Molecular studies in patients with Ogden syndrome have shown reduced N-terminal acetyltransferase protein complex A (NatA) activity and decreased N-terminal acetylation of NatA substrates. Here, NAT10 is linked to Premature aging appearance-developmental delay-cardiac arrhythmia syndrome.