An otherwise normal 48-year-old man had malignant large cell calcifying Sertoli cell tumor of the testis. There were lymph node involvement and albugineal invasion at orchiectomy, and pulmonary metastases developed despite radiotherapy and chemotherapy. Our case and, to our knowledge, the only other reported case of malignant large cell calcifying Sertoli cell tumor had clinical and histopathological features related to aggressiveness, such as unusual patient age, large tumor size, cellular pleomorphism, high mitotic rate, necrosis and aneuploid deoxyribonucleic acid. Such characteristics are not found in benign large cell calcifying Sertoli cell tumors. Associated findings included granular change of the epididymal lining, probably due to compression by the large tumor.