Aberrant activation of non‐canonical inflammasome factors has been associated with a range inflammatory pathologies; for instance, a case of a 10‐year‐old girl diagnosed with Aicardi‐Goutières syndrome (AGS), positive for heterozygous IFIH1 mutation (c.2336G > A (p.R779H)). Here, IFIH1 is linked to Aicardi-Goutieres syndrome.