KCNJ2 and Andersen-Tawil syndrome: Therefore, the diagnosis of ATS is established in individuals with two or more symptoms of the classic triad or individuals with one of the classic triad and at least one family member with ATS and/or identification of pathogenic variants in KCNJ2 and KCNJ5. Here, we report a family with ATS with a novel causative missense variant in KCNJ2 c.413A>T, p.(Glu138Val) and compare their phenotypes along with a literature review.