Spontaneous coronary dissection is rare and the diagnosis is usually post-mortem. Less than 60 cases have been diagnosed at coronary angiography. The authors report, to the best of their knowledge, the first case of multiple spontaneous coronary artery dissections in a type IV Ehlers-Danlos syndrome in a young woman admitted to hospital for acute myocardial infarction. She had a previous history of regressive complete tetraplegia due to dissection of the basilar artery and episodes of dizziness related to a dissecting aneurysm of the left vertebral artery. The diagnosis of type IV Ehlers-Danlos syndrome was established after skin biopsy had shown typical histological changes. The patient died several months later after an acute abdominal syndrome probably related to dissection of the aorta. An autopsy was refused by her family. The authors believe this to be the first case of spontaneous coronary dissection related to a type IV Ehlers-Danlos syndrome.