Leiomyosarcomas of the spermatic cord are rare tumours. The authors report the case of a 65-year-old man presenting with painful mass of the left side of the scrotum with scrotal ulceration. Left transinguinal orchidectomy and left hemiscrotectomy were performed and histological examination of the operative specimen revealed leiomyosarcoma of the spermatic cord. No metastases were observed on thoracoabdominopelvic CT. One month after discharge from hospital, the patient presented with delayed healing and complementary scrotal resection and adjuvant radiotherapy were performed. Local and regional extension is frequently observed in leiomyosarcoma of the spermatic cord, justifying radical transinguinal orchidectomy with high ligation of the cord. Cases at greatest risk of recurrence (large tumour, early local recurrence, positive surgical margins) should be identified by multidisciplinary committees and may be eligible for adjuvant radiotherapy.