Type I neurofibromatosis is associated with a dysfunctional protein resulting from the introduction of a premature stop codon in the NF1 mRNA due to false C-U editing [10]; random editing by both ADAR1 and ADAR2, as well as mutations in the APOBEC1 gene have been reported in several cancer cases [11] and ADAR mutations were identified as causative for the Aicardi–Goutières Syndrome (AGS), an inherited encephalopathy that affects newborn infants and results in severe mental and physical disability [12]. Here, NF1 is linked to Aicardi-Goutieres syndrome.