Pulmonary thromboembolism (PTE) is the major cause of maternal death in the UK. Underlying PTE is the problem of deep venous thrombosis (DVT). Inherited thrombophilia will be found in about 50% of women with a personal history of venous thromboembolism (VTE), and screening for thrombophilia should be considered in women with a personal or family history of VTE. There is currently no place for universal screening for thrombophilia in pregnancy. There are particular considerations with regard to the management of thrombophilia in pregnancy. Low-molecular-weight heparins are now the heparin of choice in pregnancy because of a better side-effect profile (substantially reduced risk of heparin-induced osteoporosis and heparin-induced thrombocytopaenia) compared to unfractionated heparin, good safety record for mother and fetus and convenient once-daily dosing for prophylaxis.