To systematically study the clinical diagnosis and treatment of smooth muscle tumor in the male reproductive system. We analyzed the ultrasonographic features, pathological findings, treatment strategies and postoperative follow-up results of 5 male patients with smooth muscle tumor in the reproductive system, and reviewed other relevant literature. Compared with leiomyoma, leiomyosarcoma exhibited stronger mixed echoes than the testis at ultrasonography, typical mitotic phase (> or = 2/10 HP) of tumor cells at HE staining, and significant expressions of HIF-1alpha and Glut-1 at immunohistochemistry. No relapse was observed in the 2 cases of leiomyoma during the 10-year follow-up after simple tumor resection, nor were recurrence and metastasis in another 3 cases of leiomyosarcoma during the first year after radical surgery without combined radio- and chemo-therapy. Primary smooth muscle tumor of the male reproductive system is difficult to be diagnosed. Ultrasonography can help to preliminarily screen leiomyosarcoma. For those with possible leiomyosarcoma, preoperative MRI and intraoperative frozen sectioning examinations are recommended for the possibility of lymphatic metastasis. Postoperative radiotherapy and chemotherapy should be chosen cautiously for those confirmed with leiomyosarcoma by pathological examination.