Abnormal liver tests occur in 3%-5% of pregnancies, with many potential causes, including coincidental liver disease (most commonly viral hepatitis or gallstones) and underlying chronic liver disease. However, most liver dysfunction in pregnancy is pregnancy-related and caused by 1 of the 5 liver diseases unique to the pregnant state: these fall into 2 main categories depending on their association with or without preeclampsia. The preeclampsiaassociated liver diseases are preeclampsia itself, the hemolysis (H), elevated liver tests (EL), and low platelet count (LP) (HELLP) syndrome, and acute fatty liver of pregnancy. Hyperemesis gravidarum and intrahepatic cholestasis of pregnancy have no relationship to preeclampsia. Although still enigmatic, there have been recent interesting advances in understanding of these unique pregnancy-related liver diseases. Hyperemesis gravidarum is intractable, dehydrating vomiting in the first trimester of pregnancy; 50% of patients with this condition have liver dysfunction. Intrahepatic cholestasis of pregnancy is pruritus and elevated bile acids in the second half of pregnancy, accompanied by high levels of aminotransferases and mild jaundice. Maternal management is symptomatic with ursodeoxycholic acid; for the fetus, however, this is a high-risk pregnancy requiring close fetal monitoring and early delivery. Severe preeclampsia itself is the commonest cause of hepatic tenderness and liver dysfunction in pregnancy, and 2%-12% of cases are further complicated by hemolysis (H), elevated liver tests (EL), and low platelet count (LP)-the HELLP syndrome. Immediate delivery is the only definitive therapy, but many maternal complications can occur, including abruptio placentae, renal failure, subcapsular hematomas, and hepatic rupture. Acute fatty liver of pregnancy is a sudden catastrophic illness occurring almost exclusively in the third trimester; microvesicular fatty infiltration of hepatocytes causes acute liver failure with coagulopathy and encephalopathy. Early diagnosis and immediate delivery are essential for maternal and fetal survival. (HEPATOLOGY 2008;47:1067-1076.) M ost pregnant women are young and healthy, and physiological changes in pregnancy must not be mistaken for liver dysfunction (Table 1). Abnormal liver tests occur in 3%-5% of pregnancies, with many potential causes (Table 2). Although relatively uncommon, any liver disease can occur coincidentally in the pregnant patient and pregnancy may occur in a patient with underlying chronic liver disease. However, most liver dysfunction in pregnancy is pregnancy-related 1 and due to one of the 5 liver diseases unique to the pregnant state-hyperemesis gravidarum (HG), intrahepatic cholestasis of pregnancy (ICP), preeclampsia, the HELLP syndrome, and acute fatty liver of pregnancy (AFLP). These conditions are complications of pregnancy itself, and each has a characteristic timing in relation to the trimesters of pregnancy: HG in the first trimester, ICP in the second half of pregnancy, and the other 3 in the third trimes...