Leiomyosarcoma of the prostate is a rare neoplasm;approximately less than 100 cases have been documented. In early studies, leiomyosarcoma was described in children and adults, but most current series suggest that prostate leiomyosarcoma only occurs in adults. Since this neoplasm is an infrequent lesion, treatment has not been well established. Most authors advise early diagnosis and emphasize the importance of radical surgery for localized disease. We report herein one case of prostatic leiomyosarcoma, in which a 27-yr-old man is still alive with no evidence of metastases 4 years post-operatively. Relevant literature of this uncommon and aggressive malignancy is also reviewed.