The Watson-Alagille syndrome is a distinctive type of congenital cholestasis, in which hepatic ductular hypoplasia is associated with various ocular, bony, and cardiac anomalies, often with a characteristic facies. The prognosis is surprisingly good, and we present a 26-year-old woman with this syndrome in whom intense pruritus and xanthomatosis had been prominent during infancy but had subsequently cleared completely. The recognition of this syndrome in a young child with cholestatic jaundice may obviate the need for a diagnostic laparotomy.