Vitamin A status was assessed in 19 patients with biliary atresia treated by hepatic portoenterostomy. Plasma vitamin A levels were almost within normal range in patients without jaundice (T.Bil2mg/dl), but significantly low both in patients with slight jaundice (2≦T.Bil<5mg/dl) and severe jaundice (T.Bil≧5mg/dl). Intestinal absorption of vitamin A was maintained in patients without jaundice and with slight jaundice, whereas significant malabsorption was suggested in patients with severe jaundice. Because liver vitamin A levels revealed over 20μg/g wet tissue in each group, hepatic reserve was considered to be adequate. After oral administration of vitamin A, the increase in RBP was smaller in patients with low plasma vitamin A than those with normal vitamin A. These results suggest that vitamin A status in biliary atresia mainly depends upon hepatic RBP production.