Persistent hyperbilirubinemia and icterus are described in a healthy 4-year-old Thoroughbred horse. Hyperbilirubinemia was not related to food intake and was not associated with evidence of increased hemolysis or with acquired hepatic disease. The hyperbilirubinemia was thought to be a result of inappropriate conjugation of bilirubin rather than any abnormality in bilirubin uptake or excretion. The bilirubinemia in this horse appears most similar to a human syndrome, caused by a familial deficiency of bilirubin-uridine diphosphate glucuronyl transferase.