Kaposiform hemangioendothelioma (KHE) is a rare vascular neoplasm associated with the Kasabach-Merritt phenomenon (KMP), which is a consumptive coagulopathy with associated potentially life-threatening thrombocytopenia. There are no standardized treatment protocols for the management of KHE with KMP. Moreover, there are limited reports regarding the treatment of cases of rebound. Herein, we describe a rare case of rebound of KHE/KMP, during systemic sirolimus treatment, successfully treated with embolization and vincristine infusion combined with microwave ablation.