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Kasabach-Merritt syndrome (KMS) is a rare and aggressive vascular tumor with thrombocytopenia and consumptive coagulopathy.However, a standard treatment regimen for KMS has not been established to date.We report a case of a 6-week-old male infant with a life-threatening Kasabach-Merritt syndrome was successfully treated with transarterial embolization and systemic corticosteroid.