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Metastatic renal cell carcinoma to the temporal bone: unusual case report. We report an extremely rare case of metastasic renal cell carcinoma to the temporal bone which presented initially as a jugulotympanic paraganglioma. The clinical and radiological appearances were misleading.Investigations of concomitant high blood pressure revealed a tumour of the right kidney. Biopsy of the mastoid mass was histologically compatible with a metastasis from a clear cell renal carcinoma.The patient underwent a radical nephrectomy and local external radiotherapy to the head. He also received adjuvant treatment with interferon-– and interleukin 2. The clinical presentation, the radiological and histological features, the patterns of spread, the treatment options and the prognosis of these tumours are discussed. A review of the literature confirms the extremely unusual occurrence of this localisation.