The hyperimmunoglobulin E syndrome was discribed for Buckley, and it also called the Job syndrome. There are two types: dominant autosomal and recessive autosomal. It is a primary, rare and complex immunodeficiency, characterized clinically by recurrent skin abscesses for Staphylococcus aureus, recurrent pneumonia, and pneumatoceles, hypereosinophylia, high serum levels of immunoglobulin E (> 2,000 Ul/mL), early eczema and late loss of primary dentition. Recently a STAT3 mutation has been described as origin of dominant autosomal hyperimmunoglobulin E syndrome. Since 1972, 250 cases have been reported around the world. The diagnosis is done with the Grimbacher criteria and the prognosis depends on the opportune diagnosis and treatment. The incidence is same in women and men. The differential diagnosis is with allergic bronchopulmonary aspergillosis, chronic granullomatose disease, T cell lymphoma, and atopic dermatitis. The treatment is with prophylactic antibiotic, intravenous immunoglobulin or recombinant INF gamma.