In this review, we will provide an overview of the current state of knowledge regarding the genetic basis of NSTA related to the EDA/EDAR/NF-κB pathway and its phenotypic overlap with HED, aiming at illustrating the importance of genetic analysis in the diagnosis of NSTA and related ectodermal disorders, and the need for ongoing research to improve our understanding of these conditions. Here, NFKB1 is linked to hypohidrotic ectodermal dysplasia.