Congenital adrenal hyperplasia (CAH) is one of the mostfrequent inborn endocrine disorders; it comprises autosomalrecessive disorders of cortisol biosynthesis in the adrenal glandcaused by various enzyme deficiencies. Because of paucity ofepidemiological data of CAH in developing countries, this studyaimed to identify clinical characters, laboratory parameters andtreatment outcomes of CAH in Egyptian children. This is aretrospective study covering 180 patients attended the DiabetesEndocrine Metabolic Pediatric Unit at Cairo University Children'sHospital being a referral center for all Egyptian governorates.Demographic data, clinical examinations, laboratory andradiological investigations and treatment options are obtained fromthe files of patients and analyzed. The results of this study showedthat deficiency of 21-hydroxylase is by far the most common causeof CAH, corresponding to more than 90% of our cases. However,3β-hydroxysteroid dehydrogenase deficiency represents 8%. Saltwasting form accounts for more than 80% of cases followed bysimple virilizing then non classic form. Parental consanguinity wasfound in 62% of cases. Genital ambiguity was the most commonpresentation followed by salt losing symptoms and signs thenpremature pubarche manifestations. Our study indicates thatnewborns with developmental anomalies of the external genitaliashould be diagnosed as early as possible so that medical,psychological, and social complications are minimized.