The jugular foramen (JF) region is a complex area of the cranial base where venous structures such as the jugular bulb and the inferior petrosal sinuses are strictly related to the lower cranial nerves IX, X and XI. The most common tumours include glomus jugulare, schwannomas of the mixed cranial nerves (IX-XI) and meningiomas. Schwannomas involving the jugular foramen are rare neoplasms and in most of the cases are thought to originate from the X cranial nerve. We report a case of a schwannoma of the JF diagnosed at an early stage, allowing radiological and surgical evidence to support its origin from the tympanic branch of the IX cranial nerve. To our knowledge this is the first case reported in the literature of such a tumour.