Intrahepatic cholestasis of pregnancy occurs mainly in the final months of pregnancy, and is characterised by pruritus. Foetal morbidity and mortality are increased. The disorder is probably caused by a genetic defect in hormonal metabolism which becomes manifest during the altered hormonal balance in pregnancy. The total serum bile acid concentration is the diagnostic hallmark of the disease. Other routine laboratory tests have a low sensitivity and specificity. An active obstetric management with routine foetal assessments, caesarean section if the foetal condition worsens, and termination of the pregnancy at 37-38 weeks, probably prevents a large proportion of complications. Ursodeoxycholic acid treatment should be considered especially in patients with severe pruritus or complications in previous pregnancies.