The liver is known to play a central role in the chylomicron metabolism. It synthesizes apolipoproteins (e.g., apo A I, apo C II, apo E), lecithin cholesterol acyltransferase, hepatic lipase and is responsible for the chylomicron remnant removal. Not surprisingly then the chylomicron metabolism is compromised in liver diseases such as acute hepatitis, cirrhosis and cholestasis. The authors reviewed the normal features of the chylomicron metabolism which are essential for a better understanding of the lipoprotein disturbance in liver diseases.