Abstract: Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor with local invasiveness. It is found predominantly in infants and children, and often accompanied by the Kasabach-Merritt phenomenon (KMP). There is no evidence-based standard for KHE treatment yet. The sirolimus is expected to be the first-line drug due to its efficacy and safety. This article reviews the progress of the diagnosis and treatment of KHE.