Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in infants and children, and primary rhabdomyosarcoma of the genitourinary tract accounts for 20-25% of cases, with approximately half of genitourinary rhabdomyosarcomas originating in the bladder or prostate. Surgery remains the primary treatment for rhabdomyosarcoma, and complete surgical resection of the tumor combined with improved chemoradiotherapy regimens has improved patient survival, with 5-year survival rates increasing from 20% initially to approximately 80% currently. However, the majority of rhabdomyosarcomas of the cystoprostate occur in children younger than 5 years of age and the tumors are located deep in the narrow pelvic cavity, increasing the difficulty of surgical resection. Currently, there is a controversy regarding the surgical approach to RMS of the bladder/prostate using radical surgery or conservative treatment with partial resection of the tumor and preservation of the bladder. Despite the continuous improvement of adjuvant therapies such as chemoradiotherapy, the long-term efficacy of conservative treatment remains to be clarified by further studies, and the long-term effects of intensification and prolongation of chemoradiotherapy in conservative treatment need to be further evaluated. Moreover, for patients with poor preoperative chemotherapy and insignificant tumor shrinkage, prolonging the dose and course of chemoradiotherapy does not necessarily improve the patient’s prognosis and may even lead to delayed growth and development, bladder wall fibrosis, and association with urinary incontinence and sexual dysfunction in adulthood. Therefore, the dose and scope of chemoradiotherapy should be limited as much as possible in children, and the long-term effects of high-dose radiotherapy on bladder function impairment need to be considered. In the Department of Urology of the Pediatric Hospital of Fudan University, a team of oncologists led by Professor Yun-Li Bi, the head of the department, has in recent years carried out radical surgical resection of tumors combined with appropriate chemoradiotherapy for the treatment of rhabdomyosarcoma of the bladder/prostate in children, with satisfactory clinical results. children, including 1 female and 13 male cases. A total of 12 patients underwent surgery after biopsy and preoperative chemotherapy, with a mean age of 3.08 years (6 months to 15 years) at the time of surgery. 8 patients underwent tumor resection with preservation of the bladder and cystourethral anastomosis, and 4 patients underwent radical cystectomy with in situ neocystoplasty. Chemotherapy was continued after surgery, and some children received radiotherapy. The mean follow-up time after surgery was 25.5 months (7 months to 4.4 years), and all patients survived during the follow-up period, and there were no patients with definite recurrence, with satisfactory clinical outcomes. Moreover, in the clinical assessment of urinary control and bladder function in postoperative children, we found that 11 of 12 patients did not develop postoperative urinary incontinence and only one patient developed intermittent daytime wetting of pants after surgery, and the symptoms gradually disappeared after biofeedback voiding training. Even with a new bladder replaced after total cystectomy, the children were able to empty urine on their own by abdominal pressure. Except for one child with total cystectomy and controlled ileocystic neobladder requiring clean catheterization, all children were able to urinate from the urethra and had no obvious residual urine on ultrasound, achieving clinically satisfactory urinary control. Our experience shows that for children with small tumors that are confined to the prostate or bladder neck, complete resection of the tumor lesion with preservation of the bladder is possible. For patients whose bladder cannot be preserved during secondary surgery after biopsy and preoperative chemotherapy, we prefer the treatment option of radical total cystoprostatectomy combined with in situ ileal neobladder, with good results in recent postoperative follow-up, with 100% of children surviving tumor-free during the follow-up period and none having significant postoperative urinary incontinence. In patients with tumor infiltration depth below the membranous urethra into the anterior urethra, the neobladder and residual urethra cannot be anastomosed, so controlled urinary diversion is required after complete resection of the tumor, and the neobladder can be emptied by its own clean catheterization after surgery and also have a satisfactory quality of life. Therefore, we believe that in children with RMS of the bladder/prostate, complete surgical resection of the tumor is the main goal of treatment and plays an important and critical role in improving prognosis and survival, while reducing the course of chemoradiotherapy and its associated side effects. In particular, radical surgery is of greater significance in patients with poor preoperative chemotherapy and insignificant tumor shrinkage.