Sarcoma of the prostate and sarcomatoid carcinoma of the prostate are rare conditions, both characterized by a poor prognosis. Sarcomatoid carcinoma of the prostate typically arises from the evolution of an underlying adenocarcinoma, occasionally featuring heterologous elements, bulky disease being possible but rare. In contrast, sarcoma of the prostate derives from non-epithelial mesenchymal components of the prostatic stroma, shows rapid growth, and frequently presents as massive pelvic tumors obstructing the urinary tract at the time of diagnosis. We report the case of a 55-year-old patient with a two-month history of symptoms of urinary obstruction. The patient presented with an extremely enlarged heterogeneous prostate, although his prostate-specific antigen level was low. The lack of a history of prostatic neoplasia led us to suspect sarcoma, and a transrectal prostate biopsy was carried out. An immunohistochemical study of the biopsy specimen did not confirm the clinical suspicion. However, in view of the clinical features, we believe that sarcoma of the prostate was the most likely diagnosis. The patient received neoadjuvant chemotherapy followed by radiation therapy. At this writing, surgical resection had yet to be scheduled.