Clear cell adenocarcinoma of the cervix (CCAC) is an uncommon tumor. No good treatment option has been reported for advanced disease, and the prognosis is generally poor. We report a case of a 14-year-old girl with stage III CCAC. She was given whole-pelvic external irradiation (40 Gy in 20 daily fractions) and high-dose rate brachytherapy with concurrent weekly cisplatin (40 mg/m(2)), followed by further external irradiation to the parametria with central shield (14 Gy in seven daily fractions). She then received one cycle of carboplatin (area under the curve [AUC] 6) with paclitaxel (175 mg/m(2)) and two cycles of carboplatin (AUC 4) with gemcitabine (1000 mg/m(2) on days 1 and 8) because she developed anaphylactic shock to paclitaxel. Chemotherapy was stopped after the third cycle due to initial poor general condition. However, she gradually improved while on palliative care. Reassessment 6 months later showed no evidence of residual disease, and she remained disease free during a follow-up of 1 year. The complete response in this case suggests that chemoirradiation followed by combination chemotherapy may be a treatment option for advanced CCAC.