DNA analysis revealed a heterozygous variant in the patient: c.823delT (p.S275fs <sup><i>∗</i></sup> 13 at protein level) in the IRAK4 gene.<h4>Conclusions</h4>The importance of clinical suspicion is emphasized in order to confirm the diagnosis by IRAK4 gene sequencing and provide the appropriate treatment for this rare primary immunodeficiency, as soon as possible. The gene discussed is IRAK4; the disease is inborn error of immunity.