Pregnancy is associated with an increased risk of venous thromboembolism (VTE), and this condition remains an important cause of maternal morbidity and mortality. Approximately 50% of gestational VTE are associated with thrombophilia. Recent studies suggest that there is also a link between thrombophilia and pregnancy loss, as well as other gestational vascular complications. Although the most compelling data derive from women with antiphospholipid antibodies, the use of anticoagulation for prevention of these complications in women with heritable thrombophilia is becoming more frequent. This article reviews the management and prevention of VTE and other complications related to the heritable thrombophilias during pregnancy, an area that remains particularly challenging because of the potential for anticoagulant-related fetal as well as maternal complications and the paucity of good-quality data upon which to base clinical decisions.