Ehlers-Danlos syndrome (EDS) is associated with easy bruising and bleeding complications in the majority. Although bleeding complications are frequently observed during surgery in these patients, the perioperative hemostatic prophylaxis of patients with EDS is not standardized. We present 2 cases of effective perioperative hemostatic management of patients with EDS and review the literature to raise awareness of hemostatic issues during surgery and discuss medical options to consider for perioperative hemostatic management based on our clinical experience and literature review.