The primary localization of hemangioendothelioma in the bone is very rare and the clinical and radiological diagnosis is often difficult. Due to the rapid growth, invasive properties and early metastatization, the evolution of these neoplasms is often progressive, despite the radical removal followed by radiotherapy. A case of a malignant hemangioendothelioma of the skull in a 6-year-old male patient is reported. The radiological aspects supported the diagnosis of fibrous dysplasia of bone. The patient was operated on, and an apparently total removal of the tumor was performed, followed by cobaltotherapy; however, the growth of the neoplasm was not arrested. Thus, chemotherapy with intravenous Enduxan (50 mg per day) was employed during a month, with clinical recovery and radiological cure of the process. Some clinical features on the differential diagnosis of hemangioendothelioma are discussed due to the confusion on the terminology and classification of these tumors.