We report a case of primary leiomyosarcoma of the testis, which was believed to originate from normal testicular structures containing smooth muscle cells, such as 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. There was no evidence of tumor after 2 years. The literature is reviewed and the therapeutic approach is discussed.