Ectodermal dysplasia (ED) is a genetic disorder which is characterized by deficient tissues from ectoderm and mesoderm. In this systematic review, two researchers searched keywords such as Ectodermal Dysplasia and Hypohidrotic Ectodermal Dysplasia (HED) in Medline (PubMed), Scopus, and Web of Science databases were searched until June 2020. In the first search 885 articles were found. After including and excluding criteria, included articles were reduced to 53 in Scopus, 11 in Web of Science, and 11 in PubMed. The point is that out of 75 selected studies, 52 articles were identified the cause of infection in ectodermal dysplasia as mutations in the NEMO gene and its subsets.