A case of embryonal rhabdomysarcoma arising from the prostate is reported and a review of literatures related to this case in presented. The patient, a 41-year-old Korean man, was admitted to Busan National University Hospital with a complaint of dysuria. The examination revealed a hard mass in left lobe of the prostate extending to prostatic urethra. The gross specimen cosisted of a prostate and a polypoid mass. The mass in left lobe of the prostate was 3×3 cm and was ill-defined. The cut surface was grayish white, fish flesh in appearance. The mass of the prostatic urethra was 2.7×1.5cm. The external surface was smooth and nodular. The cut surface was similar to that of the prostate. Microscopically, the masses were confirmed to be an embryonal rhabdomyosarcoma. Eighteen month later, the tumor was recurred.