Factor V Leiden is a common genetic mutation that predisposes its carriers to venous thromboembolism. When combined with the hypercoagulable state that is characteristic of pregnancy, there is an increased risk of severe and recurrent pregnancy complications. Factor V Leiden is the most common cause of primary and recurrent venous thromboembolism in pregnancy. Factor V Leiden carriage has consistently been shown to increase the risk of early onset gestational hypertension and HELLP syndrome (Hemolysis, Elevated Liver enzymes, Low Platelets) in pregnancy. Maternal carriage of factor V Leiden is also associated with severe placental abruption and fetal growth disturbances. Although it is unclear whether factor V Leiden causes an increased risk of first trimester miscarriage, it is associated with stillbirth and placental infarction. Patients with venous thromboembolism or severe pregnancy complications should be tested for factor V Leiden and other inherited and acquired thrombophilia. Therapeutic heparin is required for acute thromboembolic events in pregnancy. Patients with factor V Leiden and a previous venous thromboembolism may, according to their level of risk, be offered either prophylactic or therapeutic heparin. The role of antithrombotic therapy in the prevention of severe pregnancy complications remains unclear.