Loeys-Dietz syndrome (LDS) is an autosomally dominant connective tissue disorder characterised by vascular and skeletal manifestations. It is caused by mutations in the TGFBR1, TGFBR2, TGFB2 or SMAD3 genes. There are four types of which type I is the most common accounting for more than 75% of the cases. Here we report the anaesthetic management of a case of LDS type I who presented with abdominal pain with suspected twisted ovarian cyst for laparotomy.