Background: Kasabach-Merritt syndrome is an infrequent complication of large hemangiomas that could be life-threatening. Ninety percent of this phenomenon is associated with kaposiform hemangioendothelioma. Aim of the study: To present the therapeutic process of a child with kaposiform hemangioendothelioma complicated by this rare syndrome. Case report: The patient was admitted to the hospital during the first month of life due to a lesion on the head and neck. After many attempts with pharmacological treatment, surgery was the only effective treatment.Conclusions: We want to emphasize that operative management of these tumors should be considered in cases of treatment resistance or rapidly progressive growth of the hemangioendothelioma.