Summary

Primary tumours of the rete testis, either benign or malignant, are very rare. Such lesions require careful scrutiny including immunohistochemical analysis to exclude malignant lesions such as mesothelioma or papillary serous carcinoma. To the best of our knowledge this is the first report of a primary clear cell carcinoma of the rete testis. The possibility of a primary renal cell carcinoma or other primary site with metastatic spread to rete testis was excluded by thorough clinical and radiological work-up.