Background: Embryonal rhabdomyosarcoma (ERMS) of the prostate is a rare and aggressive malignant tumor in adults with a poor prognosis in general. The main presenting symptoms are dysuria and acute urinary retention. Prostate ERMS is easily misdiagnosed, which leads to delays in treatment. Case Description: We report the case of a previously healthy 31-year-old man who presented with dysuria for 3 months. Digital rectal examination (DRE), ultrasound, and computed tomography (CT) were suggestive of the possibility of a 2.0 cm x 3.0 cm abscess in the left lobe of the prostate. Prostate-specific antigen (PSA) was at a normal level, and C-reactive protein (CRP) concentration was elevated. The patient underwent surgical drainage by transurethral incision of the prostatic abscess. There was a significant improvement in the maximal urinary flow rate on postoperative day 5. However, the patient presented with acute urinary retention again on the 20th postoperative day. DRE and pelvic magnetic resonance imaging (MRI) showed a recurrence of an enlarged mass in the left lobe of the prostate. PSA level was again normal. Cystoscopy revealed a large neoplasm arising from the prostate obstructing the bladder outlet, and a diagnostic transurethral resection of this prostate tumor was performed. Histopathology revealed the mass to be an embryonic rhabdomyosarcoma. Subsequently, the patient was transferred to the Oncology Department and received chemoradiotherapy. After chemoradiotherapy, the size of the prostate became normal. Unfortunately, the patient deteriorated rapidly and eventually succumbed to multiple organ failure 1 year after the initial presentation. Conclusions: Due to its non-specific presenting symptoms, prostate ERMS can be easily misdiagnosed, which can lead to treatment delay. Multimodality approaches, including neoadjuvant/adjuvant chemotherapy, radiotherapy, and radical resection, can improve the survival rate and reduce ERMS patient mortality.