Kaposiform hemangioendothelioma, a rare vascular pediatric tumor often associated with Kasabach-Merritt phenomenon, is characterized by severe thrombocytopenia and consumptive coagulopathy. Kaposiform hemangioendothelioma is a severe disease and may progress quickly, resulting in a high mortality. However, standard treatment regimens for Kasabach-Merritt phenomenon have not yet been established. We reported here an infant with a large congenital kaposiform hemangioendothelioma in his chest wall who responded extremely well to surgical excision.