Background: Hepatic Hemangioma (HH) is described as the most prevalent benign hepatic tumor, and it is usually discovered incidentally as a symptomless mass of less than 3 cm. Kasabach-Merritt Syndrome (KMS) is described as a rare but severe complication of some vascular tumors. This syndrome presents as severe thrombocytopenia accompanied with a consumptive coagulopathy. Case presentation: A 35-year-old female, with a history of a hepatic tumor that was incidentally diagnosed with an ultrasound, later develops abdominal distention, petechiae and prominent hematomas that appear with minimal trauma. At the initial approach are found hematological data of microangiopathic anemia, thrombocytopenia and consumptive coagulopathy accompanied with the histopathological and imaging diagnosis of a Giant HH. Conclusion: Although, it is a very rare complication, the severity of this Syndrome requires a prompt diagnosis and treatment.

Keywords: Kasabach-Merritt Syndrome, Hepatic Hemangioma, Giant Hepatic Hemangioma, Thrombocytopenia, Disseminated Intravascular Coagulation, Case Report.