Tehran University of Medical Sciences Iranian Journal of Allergy, Asthma and Immunology 1735-1502 19 6 2020 12 19 667 675 Sule Haskologlu Department of Pediatrics, Faculty of Medicine, Division of Immunology and Allergy, Ankara University, Ankara, Turkey Figen Dogu Department of Pediatrics, Faculty of Medicine, Division of Immunology and Allergy, Ankara University, Ankara, Turkey Gulnur Gollu Bahadır Department of Pediatric Surgery, Faculty of Medicine, Ankara University, Ankara, Turkey Setenay Akyuzluer Department of Pediatrics, Faculty of Medicine, Division of Immunology and Allergy, Ankara University, Ankara, Turkey Ergin Ciftci Department of Pediatrics, Faculty of Medicine, Division of Infectious Diseases, Ankara University, Ankara Turkey, Ankara, Turkey Demet Altun Department of Pediatrics, Faculty of Medicine, Ufuk University, Ankara, Turkey Sevgi Keles Department of Pediatrics, Faculty of Medicine, Division of Immunology and Allergy, Necmettin Erbakan University, Konya, Turkey Meltem Kologlu Department of Pediatric Surgery, Faculty of Medicine, Ankara University, Ankara, Turkey Aydan Ikinciogullari Department of Pediatrics, Faculty of Medicine, Division of Immunology and Allergy, Ankara University, Ankara, Turkey 2020 02 17 2020 09 05 Loss-of-function (LOF) mutations in signal transducer and activator of transcription 3 (STAT3) gene causes autosomal dominant hyper immunoglobulin E syndrome (AD-HIES or Job’s Syndrome), a rare and complex primary immunodeficiency (PID) syndrome characterized by increased levels of IgE (>2000 IU/mL), eosinophilia, recurrent staphylococcal skin abscesses, eczema, recurrent pneumonia, skeletal and connective tissue abnormalities. Although bacterial and fungal infections are common in AD-HIES, susceptibility to parasitic infections has not been reported. Alveolar echinococcosis (AE), a zoonosis caused by the growth of the Echinococcus multilocularis (EM) metacestode, mimics slow-growing liver cancer. The mortality rate of AE is very high when it is diagnosed late or under-treated. Here, we report a 14-year-old boy with AE infections of the liver and the lung resulting in liver failure and diagnosed as STAT3-LOF. To our knowledge, the association between these two conditions has not been reported in the literature before. https://ijaai.tums.ac.ir/index.php/ijaai/article/view/2716