We present a 6-year-old male patient with markedly elevated IgE and severe atopic dermatitis presenting with staphylococcal bacteremia found to have a heterozygous variant in <i>FLG</i> (p.S3247X) and multiple variants of unknown significance in <i>BCL11B, ZAP70, LYST,</i> and <i>PTPRC</i>. Here, BCL11B is linked to bacterial infectious disease with sepsis.