Intracranial extradural hematoma is usually traumatic. Rarely, it can occur spontaneously associated with coagulative disorders (spontaneous or iatrogenic), dural vascular malformation, cranio-facial tumors and infections. In these cases, spontaneous extradural hematoma (SEH) is a serious event that needs to be recognized and managed in time to avoid fatal outcome. The authors report a case of a 12-year-old young girl with a 3-year history of right frontal sinusitis treated urgently for a right frontal extradural hematoma involving the orbit. Diagnosis and management of this case is discussed reviewing the pertinent literature.