IKBKG mutations are related to two clinically distinct hereditary genetic diseases, incontinentia pigmenti (IP, OMIM #308300) and anhidrotic ectodermal dysplasia with immunodeficiency (EDA-ID, OMIM #300291), depending on genetic status and their inhibitory effects on NF-κB activation. The gene discussed is NFKB1; the disease is incontinentia pigmenti.