Intestinal Epithelial Cell Dysplasia (IED), also known as Congenital Tufting Enteropathy (CTE), is a rare autosomal recessive disorder characterized by early-onset severe intractable diarrhea that may cause irreversible intestinal failure. The prevalence is still unknown, however, its observed to be higher among Arabs than other populations. IED exhibits clinical and histological heterogeneity. It is also associated with malformations or other epithelial diseases, and is related to abnormal enterocytes development and/or differentiation. Mutations in the EPCAM gene are known to cause IED.