In contrast to its disease‐causing role in AGS, ADAR1 is an exciting new immuno‐oncology target and several in vitro and in vivo studies have highlighted that its deletion increases tumor cell lethality and renders tumors more vulnerable to immunotherapy (Gannon et al, 2018; Ishizuka et al, 2019; Liu et al, 2019). This evidence concerns the gene ADAR and Aicardi-Goutieres syndrome.