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Our case is different from this classical information in the literature, since he presented with neonatal onset inflammatory bowel disease within the first months of life accompanied by deep ulcers throughout colonic mucosa. Moreover, he developed chronic lung disease during follow-up and histopathological examinations showed granulomas in both gastrointestinal tract and lung parenchyma. Genetic analysis revealed the diagnosis of IPEX syndrome with a germline mutation in FOXP3. Thus, our study provides an unusual presentation of IPEX syndrome with colitis and granulomas presence in histopathological examinations.<\/jats:p>","DOI":"10.1177\/1093526621998868","type":"journal-article","created":{"date-parts":[[2021,3,8]],"date-time":"2021-03-08T13:20:06Z","timestamp":1615209606000},"page":"252-257","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":4,"title":["New Findings of Immunodysregulation, Polyendocrinopathy, and Enteropathy X-linked Syndrome (IPEX); Granulomas in Lung and Duodenum"],"prefix":"10.1177","volume":"24","author":[{"given":"Demet Teker","family":"Duztas","sequence":"first","affiliation":[{"name":"Department of Pediatric Gastroenterology and Hepatology, Gazi University, Ankara, Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lina","family":"Al-Shadfan","sequence":"additional","affiliation":[{"name":"Department of Pediatric Pulmonology, Bezmialem Vakif 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