We present the first and unique case of a rapid-growing skull hemangioma in a patient with Klippel-Trénaunay-Weber syndrome. This case report provides evidence that not all rapid-growing, osteolytic skull lesions need to have a malignant character but certainly need a histopathological verification. This material offers insight into the list of rare pathological diagnoses in an infrequent syndrome.
Keywords: Hemangioma; Klippel–Trénaunay–Weber syndrome; Skull hemangioma; Skull tumor; Surgical pathology.