This review describes the features of glomus jugulare tumours with metastases. There were 100 sites of metastasis in the 53 cases previously reported. The sites of metastasis may be summarized as bone (33), lungs (23), lymph nodes (19), liver (nine) and other (16). Metastases presented up to 30 years after the initial treatment. The mean age of patients was 45 years and the sex ratio was approximately two females to one male, with no significant difference compared to non-metastatic tumours. There was a significantly higher incidence of pain and a significantly lower incidence of hearing loss at presentation compared to non-metastatic tumours. The commonest treatment was a combination of surgery and radiotherapy. The duration of symptoms before diagnosis was significantly shorter and the rates of persistent or recurrent local disease and death were significantly higher than for non-metastatic tumours. This review highlights the fact that glomus jugulare tumours are not always benign.