A 50-year-old male with paratesticular leiomyosarcoma was reported. Clinically, the pa-tient presented with a palpable, painless mass in the left scrotum for several years. Rapid in-crease in size of the mass was noted by the patient n recent months. Ultrasound for scrotum re-vealed a paratesticular mass with heterogeneous echogenicity. CT scan and MRI for the pelvis confirmed the lesion and suggested the possibility of paratesticular sarcoma. Radical orchiec-tomy with early clamping of spermatic cord was performed. The final pathological report was high-graded leiomyosarcoma of spermatic cord. After six months, the follow-up revealed no evi-dence of local recurrence or distant metastasis. We presented ths rare case of paratesticular leiomyosarcoma and reviewed the relevant literature. (J Urol R.O.C., 8:152-156,1997)