We describe a woman with Ehlers-Danlos syndrome and aortic aneurysm who experienced a myocardial infarction due to spontaneous left circumflex coronary artery dissection 3 weeks postpartum. She developed end-stage heart failure and subsequently underwent a successful orthotopic heart transplantation. To our knowledge, this is the first report of a heart transplant performed in an individual with Ehlers-Danlos syndrome.