This report describes an unusual case of uterine cervical Wilms tumor treated successfully without hysterectomy or radiation therapy. The 12-year-old white girl developed a persistent vaginal discharge. Her pelvic examination revealed a large mass involving the entire upper vagina, obscuring the cervix. Biopsy of the mass was consistent with Wilms tumor with favorable histology. The tumor was not initially resected because the resection would involve hysterectomy and partial resection of the bladder wall. The patient was treated with preexcisional chemotherapy consisted of alternating vincristine, doxorubicin, cyclophosphamide and carboplatin/etoposide. Repeat magnetic resonance imaging after 5 weeks of chemotherapy demonstrated marked reduction of the tumor size. The tumor was easily removed by transsection of the stalk followed by cold-knife conization of the cervix. The patient received four more cycles of chemotherapy and remained in complete remission 12 months after completion of chemotherapy. This report suggests that in selected cases, chemotherapy can reduce tumor size sufficiently in patients with bulky cervical Wilms tumor to allow local resection and avoid hysterectomy.