This study retrospectively analyzed the clinical data of 1 case of spermatic cord leiomyosarcoma admitted to the urology department of the Affiliated Hospital of Chengde Medical College. The clinical characteristics, diagnosis, treatment and prognosis was discussed with the literature review. Radical resection of the left testicle and high ligation of the left spermatic cord were performed. Postoperative pathology was spermatic cord leiomyosarcoma. Its clinical manifestations are painless masses, which are mainly confirmed by pathological examination. The treatment is mainly radical resection, and postoperative radiotherapy can improve the prognosis and reduce recurrence.