Objective To explore the diagnosis and treatment of Kaposiform hemangioendothelioma (KHE),deepen the understanding of KHE and Kasabach-Merritt phenomenon (KMP),and discuss the optimal treatment for KHE.Methods From January 2008 to August 2016,13 cases of KHE were confirmed by surgery or biopsy pathology and admitted to the First Affiliated Hospital of Sun Yat-Sen University,and the clinical diagnosis and treatment were analyzed retrospectively.Results There were 13 patients(7 males and 6 females) with a median age of 1.0 years(0.2-10.0 years),and 84.6%(11/13 cases) were infants and young children,76.9% (10/13 cases) involved with deep tissue,23.1% (3/13 cases) were associated with KMP and they were younger than 1 year old,and 15.4% (2/13 cases) coexisted with hemangioma or lymphangioma.The location,extent and infiltration depth of the lesion were observed by imaging examinations and histopathology showed nodule shaped spindle tumor cells.Radical resection was considered if possible.Dose of Vincristine (0.5 mg/m2 weekly) and Propranolol [1 mg/(kg·d)] were administered.The prognosis was different in thirteen cases undergoing different treatments.After 3 months to 9 years follow-up,41.7% (5/12 cases) survived after tumor treatment.Conclusions KHE happens mostly in infants and young children,with varying clinical manifestations and a high recurrence rate.The diagnosis of KHE is based on histological examination,computed tomography and magnetic resonance imaging while it still need explicit pathological diagnosis is needed.KHE may be accompanied by hemangioma or lymphangioma.Prognosis is affected by many factors and the comprehensive treatment is required.KMP should be remedied preferentially,individual treatment protocol and long term follow-up are necessary.