With the advanced development of genetic testing technology, 7 related pathogenic genes have been revealed thus far, including TREX1 (22%), RNASEH2B (36%), RNASEH2C (12%), RNASEH2A (5%), SAMHD1 (13%), ADAR1 (6%), and IFIH1 (3%), corresponding to AGS types 1–7, respectively [4]. Here, RNASEH2B is linked to Aicardi-Goutieres syndrome 1.