We report a case of proven thyroid medullary carcinoma, metastatic to the temporal bone. Review of the otolaryngology literature demonstrates that this is the first such report. The patient presented with symptoms, physical examination, and imaging studies suggestive of a glomus jugulare tumour. The pre-operative diagnosis of metastatic thyroid medullary carcinoma, however, was made, based on a history of medullary carcinoma of the thyroid with previous metastases as well as an elevation in calcitonin levels. Histological examination of the surgical specimen confirmed the diagnosis.