ABSTRACT
BACKGROUND: Alagille 's syndrome is the main syndromic chronic intrahepatic cholestasis characterized by hypoplasia of the intrahepatic bile ducts. It is a multisystem disorder of autosomal dominant inheritance with involvement of multiple organs. Usually it becomes apparent in the neonatal period, presenting as subclinical cases or severe degrees of the disease with the consequent development of liver cirrhosis and subsequent liver failure associated with multiple abnormalities: defects in the vertebral arches, typical facies, pulmonary stenosis, mental retardation and hypogonadism. OBJECTIVE: To present the first case of partial external biliary diversion in Argentina, showing the surgical technique and the improvement in the quality of life, as an alternative to be considered in patients with Alagille's syndrome before the development of cirrhosis. RESULTS: It has been shown that partial external biliary diversion can stop the process of liver fibrogenesis, halting the progression of the disease and avoiding the need for transplantation in some types of intrahepatic cholestasis when cirrhosis has not been established. DISCUSSION: This surgical technique can improve the quality of life and morbidity associated with hypercholesterolemia in patients with Alagille's syndrome, delaying and maybe avoiding the need for liver transplantation.