Ehlers-Danlos syndrome is associated with fatal cardiovascular complications and intraoperative death. The lack of literature on cardiac surgery in the syndrome makes it difficult for surgeons to assess the risk of an operation and to choose optimal treatment. We describe the case of a 63-year-old man with Ehlers-Danlos syndrome type I or II, mitral regurgitation and atrial fibrillation who was managed successfully with mitral valve repair and surgical cardioversion.