Intrahepatic cholestasis of pregnancy is pregnancy-specific liver disorder, characterized by pruritus as the main clinical symptom, and fasting liver function tests. The term thrombophilia is used to describe a group of conditions characterized by blood coagulation disorder with increased risk of blood clot formation, which may be congenital or acquired. In general, population the incidence of thrombophilia and intrahepatic cholestasis of pregnancy varies widely, depending on the type of disorder (in case of congenital thrombophilia) and geographical distribution (in case of intrahepatic cholestasis of pregnancy). A high incidence of pregnancy complications makes both congenital thrombophilia and intrahepatic cholestasis of pregnancy very important in clinical practice. At the same time, association between these two disorders is extremely complicated in management, due to perinatal risks. The key-point for the management is cooperation among obstetricians, hematologists, and hepatologists, being crucial for better outcomes. This is an open access article distributed under the terms of the Creative Commons Attribution License (CC BY 4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.