Nuclear factor kappa B essential modulator (NEMO) results in X-linked ectodermal dysplasia with immunodeficiency due to its hypomorphic mutations.1 In our case, as both of the patient's parents and her siblings (two sisters and a brother) were clinically unaffected, it suggests that she had either autosomal recessive inheritance or a de novo autosomal dominant mutation. The gene discussed is IKBKG; the disease is Immunodeficiency.