Herein we report a case of primary leiomyosarcoma of testis, which was believed to originate from normal testicular structures. It contained smooth muscle cells, blood vessels and contractile cells of the seminiferous tubules. No evidence of tumor spread was found.Treatment consisted of orchiectomy with high ligation of the spermatic cord. The patient received no adjuvant therapy and there was no evidence of tumor 30 months after the operation. Pertinent literature is reviewed and the differential diagnosis is discussed.