Klippel-Trenaunay syndrome (KTS) is a complex congenital disorder characterized by a triad of varicose veins, cutaneous capillary malformation, and hypertrophy of bone and/or soft tissue. KTS may be associated with massive hemorrhage or coagulopathy that be a life-threatening situation. Although women in reproductive age are at risk of ruptured corpus luteum with active arterial bleeding, if it managed properly, the development of serious complications, such as disseminated intravascular coagulation (DIC) rarely develops. However, in case of patient with vascular malformation, there is possibility of unexpected complication occurrence such as DIC. The authors report a case of a 29-year-old female with KTS who presented with corpus luteal hemorrhage and which lead to DIC, despite adequate surgical and medical treatment.