Sterile alpha motif and HD domain-containing protein 1 (SAMHD1) was initially associated with Aicardi-Goutières Syndrome (AGS) and has been identified as a deoxyribonucleotide triphosphohydrolase (dNTPase) with a well-defined human immunodeficiency virus type one (HIV-1) restricting function [14,15]. Here, SAMHD1 is linked to Aicardi-Goutières syndrome.