Large cell calcifying Sertoli cell tumor of the testis (LCCSCT) is a rare tumor that is usually benign and multifocal. It may be associated with hereditary endocrine anomalies such as Carney's and Peutz-Jeghers syndromes. It is a rare histological variant of sex cord stromal tumors. It is exceptional in elderly men and the outcome is rarely fatal. We report a case of LCCSCT in a 44 year-old man with fatal outcome. The tumor involved the right testis and several areas of the tunica albuginea were grossly invaded. It composed of cords and trabeculae of large polygonal cells embedded in a myxoid and fibrous stroma with areas of calcification along with nuclear atypia, necrosis, and abundant mitoses. The Peutz-Jeghers syndrome (PJS) is known to be commonly associated with ovarian tumors. However, its association with testicular tumors is uncommon. To the best of our knowledge, this is the eight such case being reported in the literature. Our case, to our knowledge, is the only other reported case of malignant large cell calcifying Sertoli cell tumor with clinical and histopathological features related to aggressiveness, such as 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.