Clear cell adenocarcinoma of cervix is a very rare malignancy of the uterine cervix. The etiology and pathogenesis are unclear. Clear cell adenocarcinomas have been reported most commonly in women with a history of in utero exposure to diethylstilbesterol (DES), these tumors also can develope in the absence of exposure to DES. These tumors account for 4% to 9% of adenocarcinomas of the cervix. We report a case of clear cell adenocarcinoma in the uterine cervix of 40 years-old women who was not related to DES with a brief review of literature.