After uncovering Ogden syndrome in 2011, reports of different nonlethal NAA10 variants in males as well as mostly de novo variants in affected female index cases with nonspecific ID have been published, expanding the clinical spectrum and elucidating the underlying complexity of NAA10 deficiency (see Figure 4). The gene discussed is NAA10; the disease is Premature aging appearance-developmental delay-cardiac arrhythmia syndrome.