During use of home parenteral nutrition, chronic abnormalities on liver function tests are reported to occur in both children and adults at a rate ranging from 15% to 85% (1–6). The pathogenesis of home parenteral nutrition-related liver disease, which presents mainly as chronic intrahepatic cholestasis with or without jaundice, is multifactorial and involves patient-dependent factors, especially the short-bowel syndrome (2, 5–7), and nutrition-dependent factors, such as intravenous hyperalimentation (1, 8). Other factors involved are intestinal bacterial overgrowth and translocation (9–10) and disruption of the enterohepatic bile acid pool with the occurrence of deconjugated toxic bile acids (1, 8). During the early months of home parenteral nutrition-related liver disease, histologic abnormalities consist of canalicular cholestasis and portal inflammation with or without fatty infiltration. Later, severe histologic changes—namely, extensive portal fibrosis or cirrhosis—have been anecdotally reported; over months and years, these conditions lead to liver failure and death (2, 5–6, 11–13).