Metastatic spermatocytic seminoma is an extremely rare disease. No chemotherapeutic regimen is established and the use of adjuvant radiation therapy after orchiectomy as frequently performed for classical seminoma is unclear. We report the third case of proven spermatocytic seminoma metastatic in the literature and provide a review of the literature. Administering BEP polychemotherapy as in classical seminoma was poorly effective in reducing the retroperitoneal mass in a patient with spermatocytic seminoma. A review of the literature showed that all the three described patients developing metastatic disease did not receive adjuvant radiation therapy after orchiectomy, which might be considered in high-risk cases.