Extracerebral masses involving the skull base in children and adolescents are rare. We report on a 4-month-old infant who was diagnosed with a large extracerebral mass in the anterior fossa. Subtotal resection to prevent impending blindness was complicated by almost fatal bleeding. Histopathologically, vessel malformation, epitheloid hemangioendothelioma, and angiosarcoma were possible diagnoses. The residual tumor regressed spontaneously and completely within 6 months after surgery. Retrospectively, histologic and radiologic findings and clinical course prompted the diagnosis of an infantile hemangioendothelioma. To our knowledge, this is the first report on an infantile hemangioendothelioma of the skull base.