Severe cholestasis associated with long-term home parenteral nutrition is rare, and no treatment is known to be effective. This study shows a case of a patient who developed jaundice while receiving long-term home parenteral nutrition. Causes of jaundice, other than the patient's parenteral feedings, were excluded. The patient's jaundice did not respond to alterations in his parenteral feeding program or to metronidazole. Ursodeoxycholic acid (600 mg/day) led to a prompt and sustained improvement in his hyperbilirubinemia. When ursodeoxycholic acid was stopped, the patient again became jaundiced, but this resolved with reinstitution of ursodeoxycholic acid. This case suggests that ursodeoxycholic acid may be an effective treatment for home parenteral nutrition--associated cholestasis and should be evaluated further in patients with cholestasis associated with parenteral feeding.