A case of sarcoma botryoides in a 3-year-old boy with a more than 7 year survival so far after total cystectomy and bilateral ureterosigmoidostomy lead to review the literature of this type of tumor. The indication for the surgical procedure is based on the clinical findings only and does not require histological confirmation. Primary total cystectomy is considered the treatment of choice. With this management 21 out of a total of 152 cases of sarcoma botryoides reported in the literature are alive after total cystectomy and supravesical urinary diversion. Local excision of the tumor as well as radio- and chemotherapy are regarded as unsuccessful in the treatment of sarcoma botryoides.

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