This study reports on a case of an adult patient with embryonal rhadomyosarcoma of the prostate and comprehensively reviews the world literature on this subject. The patient was a 49-year-old man with advanced stage disease, who initially underwent transurethral tumor resection and after diagnosis was given courses of chemotherapy prior to undergoing radical cystoprostatevesiculectomy. The tumor underwent histological examination supplemented by immunohistochemical analysis. After surgery, the patient experienced a huge pelvic recurrence and eventually died of disease 1 year after the original diagnosis. The world literature was reviewed based both on a PubMed/Medline search and the reference lists of all the available publications on this subject and only 24 cases of primary embryonal prostatic rhabdomyosarcoma have been found in the world literature in adult males (≥18 years). Embryonal rhadomyosarcoma of the prostate in adults is a very rare and aggressive disease. The long-term disease-specific survival rate is poor. Stage influences the outcome. Early diagnosis and complete surgical resection offer patients the best chance of improved survival.