<h3>Introduction</h3> Hyper IgE syndrome (HIES) is a rare genetic immune deficiency disorder affecting multiple organs. Recurrent skin infections, abscesses, atopic dermatitis, and hypereosinophilia are common. Mutations in STAT-3 gene is associated with HIES. We present a novel heterozygous point mutation in STAT-3 gene in a patient with hyper IgE and recurrent abscesses. <h3>Case Description</h3> A 22-year-old male presented with severe atopic dermatitis and recurrent abscesses. Initial labs revealed very high serum IgE 41,559 U/ml and eosinophil count of 1290/cmm. CBC showed mild anemia. Infectious work up was all negative. Patient had normal CMP, troponin, spirometry, tryptase, Vit B12 and ECHO. Skin biopsy was suggestive of eosinophilic fasciitis. Bone marrow biopsy and flow cytometry were normal. CT scans showed inguinal and axillary lymphadenopathy and borderline hepatomegaly. He was hospitalized multiple times for I/D of his recurrent abscesses. He was treated with systemic steroids and started on Bactrim prophylaxis. In addition, Dupilumab was started for his eczema which improved his skin. He longer has abscesses. After Dupilumab his IgE level significantly decreased from >50,000 U/ml to 14,790 U/ml. HIES panel revealed a novel heterozygous point mutation in STAT-3 gene, Exon 19 c1673G>A (p.Gly558Asp). Due to patient's supporting clinical phenotype HIES was suspected. Further genetic and functional studies to confirm this variant are planned. <h3>Discussion</h3> HIES is a complex disease, and it is challenging to confirm the diagnosis. Several mutations in STAT-3 have been described but more studies are still warranted to identify different mutations. Dupilumab may improve patient's atopic dermatitis in suspected cases.