Byler's disease is an autosomal recessive condition characterized by intrahepatic cholestasis, progressive fibrotic changes and finally cirrhosis that leads to death during childhood. This is a report of a six-year-old girl with Byler's disease and retrobulbar hematoma as a result of trauma who underwent enucleation and implantation. This case report describes the anesthetic features of a patient with Byler's disease in which anesthetic agents with no or minimal hepatotoxic effect should be used to avoid deterioration of liver function.