Intrahepatic cholestasis of pregnancy is the most common liver disease during pregnancy. The main features are maternal pruritus, in the absence of a rash, and raised serum bile acids. Intrahepatic cholestasis of pregnancy is associated with an increased risk of adverse perinatal outcomes, including preterm birth, meconium-stained amniotic fluid, fetal asphyxia with neonatal unit admission or even stillbirth. The main treatment option is ursodeoxycholic acid. We review the etiology, diagnosis, adverse pregnancy outcomes, focusing especially on management of this intriguing condition.