Glomus jugulare tumours are classically described as benign tumours with a long time course often measured in decades. Although these tumours may be locally invasive, most cases are histologically benign and metastases are rare. The case of a malignant glomus jugulare tumour with a particularly aggressive pattern of spread is presented. At the time of surgery, which was within 12 months of the development of symptoms, intracranial spread and metastasis to cervical lymph nodes had already occurred, demonstrating that glomus jugulare tumours are not always benign.