The gold standard treatment of KMS is the removal of the underlying hemangioma, which will correct the consumptive coagulopathy. Although many nonoperative strategies have been used to control the disease,2,3the only curative treatment remains the surgical resection of the hemangioma. However, surgical resection of hepatic hemangiomas could be hazardous in the presence of uncontrolled coagulopathy secondary to disseminated intravascular coagulation. Perioperative substitution of consumed clotting factors with fresh frozen plasma or platelet concentrates may prevent perioperative bleeding. Hemangiectomy by means of enucleation of the tumor through dissection in a fibrous cleavage plane between the capsule of the hemangioma and surrounding normal liver parenchyma is often preferred rather than anatomical hepatic resections.4This technique, when feasible, avoids resection of normal liver tissue and minimizes damage to biliary and vascular structures.5Finally, a meticulous surgical hemostasis is paramount to prevent postoperative bleeding in patients with this syndrome.