Prostate leiomyosarcoma is reported to be accounted for 0.1% of all prostate malignancies. It is a rare, aggressive tumor with fewer than 200 cases reported [1]; hence, treatment guidelines are not well established. It was accompanied by non-specific symptoms similar to those associated with benign prostatic hyperplasia. Our patient reported having recurrent episodes of hematuria. We present a rare case of prostate leiomyosarcoma treated with chemotherapy and a literature review that discusses clinical features, diagnostic methods, and therapeutic options.