This a descriptive study of the clinical profile of patient presenting with cholestatic disorders of infancy through reviewing the medial record of the Pediatric Hepatology Unit at Cairo University Children's Hospital during the period from June 2009 to June 2010. In our study 134 infants suffering from cholestasis presented at age from (4-270) days to our medical attention.Jaundice was the most frequent symptom, representing (97%) of all symptom, biopsy was done for 98 cases in which the neonatal Hepatitis was the most frequent diagnosis (36.6%).EHBA was the most common diagnosis (28.4%) of cases.