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An infant with a large occipital hemangioendothelioma with thrombocytopenia, anemia, and hypofibrinogenemia--Kasabach-Merritt syndrome--was reported. The case, a male neonate is unique, for this is the first report with this syndrome in whom the large hemangioma was noted at birth on the midocciput simulating the occipital encephalomeningocele. With the development of thrombocytopenia of 84,000 per mm3, hypofibrinogenemia of 92 mg/dl, anemia (erythrocyte 193 X 10(4) per mm3, hemoglobin 5.9 g/dl, hematocrit 16 vol%), hepato-splenomegalia, enlargement and bluish discoloration of the tumor noted on the 21/2 months of life, total excision was intended prior to the expected occurence of the systemic purpura. The patient received fresh whole blood transfusion immediately prior to surgery, and the total excision was successfully performed. Excessive bleeding was not encountered. Abrupt rise in the platelet count, red blood cell count, hemoglobin and hematocrit to normal range was noted at the first postoperative day; he was discharged on the 17th day after surgery. Nineteen months' follow-up showed normal hematologic findings with good somatic and mental development. The specimen weighing 250 g. revealed benigh hemangioendothelioma. Silver impregnation demonstrated lobular aggregates of small vascular channels. Papillary projection of interstitial cells into the lumen, reaction of the endothelium of the vessels, newly formed thrombus, ishemic necrosis and hemorrhage, hyaline degeneration of interstitial tissue were noted. These findings suggested the disseminated intravascular coagulation within the tumor followed by fibrinolysis accounts for loss of blood corpuscles, platelet, fibrinogen and clotting factors, which leads ultimately to the consumption coagulopathy and diffuse bleeding.