Clear cell carcinoma arising in a cesarean section scar is extremely rare. Due to the rarity of the tumor, treatment strategies still need to be further elucidated. We report a case of a woman with a clear cell carcinoma of 10 cm outside the abdominal cavity in her cesarean section scar. Staging surgery revealed two lymph nodes with metastatic dissemination of a clear cell adenocarcinoma. After staging surgery, six cycles of adjuvant chemotherapy with carboplatin/paclitaxel were performed. The patient was disease-free 10 months after completion of chemotherapy. Comprehensive treatment consisting of radical surgery combined with adjuvant chemotherapy can be considered for this uncommon tumor entity.