In total, we diagnosed five cases of AGS, one case of stimulator of interferon genes (STING)-associated vasculopathy with onset in infancy (SAVI), one case of spondyloenchondrodysplasia with immune dysregulation (SPENCD), and one case of PRAAS/CANDLE. The gene discussed is STING1; the disease is Aicardi-Goutieres syndrome.