In this study, we report the clinical-neuroradiological features of two patients with AGS-like (Patient A) and CADASIL-like (Patient B) phenotypes who carried the heterozygous likely pathogenic c.407C > T (p.A136V) and c.520A > G (p.R174G) TREX1 missense variants, respectively. The gene discussed is TREX1; the disease is Aicardi-Goutieres syndrome 1.