Kaposiform hemangioendothelioma (KHE) is a rare, potentially life-threatening vascular tumor. More than 50% of cases are diagnosed within the first year of life and are often associated with a thrombocytopenic coagulopathy known as Kasabach-Merritt phenomenon (KMP). Approximately 10% of patients die because of this disease, either due to local growth or KMP. Given the rarity of the entity, there have been no prospective studies regarding the treatment of KHE or standardized outcome measures to outline a standard treatment. We describe the case of a female child diagnosed at three months of age with an intraabdominal KHE associated with KMP. She received continuous treatment with vincristine, propranolol, and prednisolone for 10 months until the disappearance of the lesion in images and resolution of the coagulopathy. As a result, she obtained complete remission and now, five years after finishing the treatment, she continues symptom-free.