Hepatic Kasabach-Merritt syndrome (KMS) is a highly complex and potentially fatal complication of severe hepatic hemangioma. Although hepatic KMS is rare,the death rate of victims is significant because it is often misdiagnosed upon presentation and thus receives improper treatment.Presently the literature lacks a systematic review of hepatic KMS.We have summarized its pathogenesis,diagnosis, systematic treatment, prognosis and outcomes in order to increase awareness of this deadly disease.