Liver transplantation in a child with severe hypercholesterolaemia in Alagille syndrome.

Introduction: Liver transplantation is a curative treatment modality in children with end stage liver disease in Alagille syndrome.
Clinical Picture: We report a 3-year-old child with this condition who had severe hypercholesterolaemia, pruritus and extensive xanthomatosis.
Treatment: Liver transplantation was performed in this patient.
Outcome: He recovered well with normalisation of his lipid profile. This procedure also resulted in resolution of the disfiguring xanthomatosis.