Inherited antithrombin deficiency (ATD) is associated with a high risk of venous thromboembolic complications. Association of ATD with other conditions such as pregnancy obviously increases thromboembolic risk and may require anticoagulant therapy for prevention. Although there are several/heterogenous international guidelines regarding thromboprophylaxis in pregnant patients with ATD, data on anticoagulant prophylaxis in this context are scarce in the literature. Thus, this situation remains a challenge both in the antepartum period and during delivery. Physicians from the French Society of Thrombosis and Haemostasis (SFTH) performed a review of the literature to suggest propositions regarding the management of thrombosis prevention based on anticoagulation and antithrombin substitution in ATD pregnant women. In this review, after reporting the thrombotic risk associated with ATD, the indication of anticoagulant therapy, its dosing regimen and monitoring, and the indication of antithrombin concentrates during pregnancy and the postpartum period are discussed as well as peripartum management. Finally, this work confirms the complex management of thrombotic prevention in pregnant patients with ATD. Indeed, it requires to take into account a multiplicity of features cited in our propositions that will hopefully provide some help in this field. This work also highlights the importance of multidisciplinary discussions for pregnant women with ATD who should be counseled in an expert center including hematologist, obstetrician, and anesthetist to optimize their management.< Réduire