Among the rhabdomyosarcomas, which represent an uncommon neoplasm of mesoderm origin comprising about 1% of all female genital malignancies, sarcoma botryoides (SB) is an embrional variant usually found in the vagina of infants and young children under 16 years of age. The onset of SB from the uterine cervix is very rare with a peak incidence in the second decade; only a few cases have been published in the international literature so far. The authors report a case of embryonal rhabdomyosarcoma of the uterine cervix which occurred in a young woman treated by radical surgery, followed by adjuvant chemotherapy and radiotherapy.