Any recessive immunodeficiency (ID), with or without ectodermal dysplasia (EDA), in which the cause of the disease is mutation in the IKBKG gene. ID/EDA-ID patients, always males, are hemizygous for an IKBKG (NEMO) mutation that preserves residual NF-κB activation (hypomorphic mutations) and may also present with osteopetrosis and lymphoedema (OL-EDA-ID). [ http://www.ncbi.nlm.nih.gov/pubmed/35163099 https://orcid.org/0000-0002-7437-8060 http://www.ncbi.nlm.nih.gov/pubmed/11047757 ]