Cholestatic liver disease in infancy is caused by a wide range of condition including genetic, metabolic, obstructive and less commonly infective causes. This is a retrospective study of the clinical profile of patients presenting with cholestatic disorders of infancy through reviewing the medical records of the Pediatric Hepatology Unit at Cairo University Children's Hospital during the period from 1st January to 31st of December 2006. In our study, Infants suffering from cholestasis presented at a mean age of 3.53+5.39 (range 0-10) months to our medical attention. The mean age at onset of symptoms was 1.85+4.27 months (range 0-12). Jaundice was the most frequent symptom, representing 98.9% of all symptoms. EHBA was the most common diagnosis (17%) of cases.