Clear cell cervical adenocarcinoma (CCA) is a rather rare malignancy of the genital tract. We report six cases of CCA, diagnosed in our laboratory during a 15-year period: five patients with sporadic primary CCA and one young patient with CCA and a history of in utero exposure to DES. The possible DES exposure, clinicopathological findings as well as the differential diagnosis and the the prognosis of such patients are presented in a mini-review of the literature.