Tehran University of Medical Sciences Immunology and Genetics Journal 2645-4831 2 4 2019 12 01 213 220 Tooba Momen Department of Allergy and Clinical Immunology, Isfahan University of Medical Sciences, Isfahan, Iran. Abdolvahab Alborzi Clinical Microbiology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran. Mohammad Hossein Eslamian Allergy and clinical immunology division, Department of pediatrics, Hamadan University of medical science, Hamadan, Iran. Jean-Laurent Casanova St Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, Rockefeller University, NY, USA. Capucine Picard Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U980, Necker Medical School, Paris Descartes Uni-versity, Sorbonne Paris Cité, France. AND Study Center for Primary Immunodeficiencies, AP-HP, Necker EnfantsMalades Hospital, Paris, France 2019 10 19 NEMO (NF-κB essential modulator) is a regulatory factor involved in signaling pathways of innate and adaptive immune system. Hypomorphic mutation of IKBKG gene on X chromosome leads to X-linked recessive anhidrotic ectodermal dysplasia with immunodeficiency. The affected boys presented developmental phenotype with hypotrichosis, hypohidrosis; hypodontia with conical incisors; and susceptibility to pyogenic bacteria, mycobacteria, and viruses. Most of them have impaired antibody response to polysaccharide antigens. Here, we presented the case report of 7 years old Iranian boy with NEMO-deficiency and unusual Aspergillus infection. https://igj.tums.ac.ir/index.php/igj/article/view/40 https://igj.tums.ac.ir/index.php/igj/article/download/40/29