Testis leiomyoma is a very rare neoplasm. It is very difficult to perform an exact preoperative diagnosis; only histological examination can prove the presence of a leiomyoma. In our case radical orchidectomy was performed because of complete substitution of normal parenchyma, the extreme rarity at long-term follow-up of a reported benign intratesticular neoplasm, and the imperfect sensitivity of the extemporary histological examination.