Ehlers-Danlos syndrome (EDS) is a rare, genetically transmitted connective tissue disorder which occurs with an incidence of 1 in 5000. Nine subtypes of EDS have been identified, with type IV being associated with sudden death due to rupture of the bowel, uterus, or major blood vessels. We describe a case of a patient with type IV EDS who presented at 30 weeks' gestation with preterm labor which was treated with subcutaneous terbutaline followed by oral maintenance therapy. Approximately 72 hours later she suffered a myocardial infarction, which led to coronary artery dissection and death. This is the first case of type IV EDS in pregnancy that resulted in maternal death due to coronary artery dissection. We suggest that betamimetic tocolytics should be avoided in patients with EDS and preterm labor.