Biochemical indicators of vitamin A status were measured in 24 children (1 month to 6 years old) with severe cholestasis starting early in life and in 21 children (3 months to 13 years old) with liver disease but without cholestasis. Liver vitamin A concentrations, expressed as micrograms of retinol per gram of liver (mean ± S.D.), were 6.3 ± 7.1 (range: 0.14 to 28) and 143 ± 108 (range: 18 to 424), respectively, in cholestatic and noncholestatic children. In infants < 6 months of age, liver vitamin A values < 10 μg per gm were found in 14 of 17 cholestatic children but in none of 3 noncholestatic subjects. Plasma vitamin A values, expressed as micrograms of retinol per deciliter (mean ± S.D.), were 23 ± 18 (range: 3 to 62) and 46 ± 33 (range: 14 to 125), respectively, for the two groups. Plasma retinol values < 10 μg per dl were always associated with liver concentrations < 10 μg per gm. Plasma retinol-binding protein was only reduced to 71% of control values in cholestatic children. The fatty acid composition of liver retinyl esters was unaffected by any condition studied. Infants with chronic cholestasis are in a precarious nutritional status very early in life relative to liver reserves of vitamin A. Plasma vitamin A values, unless < 10 μg retinol per dl, are poor indicators of inadequate vitamin A status.