Loeys-Dietz syndrome is a rare autosomal dominant connective tissue disorder. Pregnant women with Loeys-Dietz syndrome are at increased risk of serious vascular and visceral complications, including aortic dissection and uterine rupture. Multidisciplinary tertiary management aims to mitigate such complications by preconception counselling and vascular assessment, medical therapy, regular echocardiography in pregnancy and joint decision-making re-mode and timing of delivery. We report an in vitro fertilisation twin pregnancy in a woman with Loeys-Dietz syndrome first seen at our institution at 26 weeks' gestation. After monitoring via serial echocardiograms, caesarean delivery occurred at 30 + 1 weeks' gestation to allow planned delivery with suspected fetal growth restriction before uterine distension was considered an indication. The patient was discharged on Day 9 with a planned early aortic root replacement due to an increase in diameter from 39 to 43 mm, followed by the discharge of twin boys at term equivalent.