Abstract: Clear cell adenocarcinoma of the cervix（ＣＣＡＣ）, tending to occur in young women，is a rare uterine cervical neoplasms. Different from common cervix cancer, CCAC may not relate with high-risk HPV infection. It has a strong association not only with a history of exposure to diethylstilbestrol （ＤＥＳ） in utero but also other factors, and is characterized by slower growing while early metastasis, deep infiltration and poor prognosis. Patients with CCAC commonly present with an abnormal vaginal bleeding or discharging. According to the lower frequency of abnormal cervical cytology, it is difficult to diagnose CCAC in early stage. Diagnosis is based on histologic confirmation with immunohistochemical information. Up to now there has not been a standard treatment for CCAC, most patients are treated surgically for early stage, while chemoradiation with platinum-based chemotherapy for late stage. Operation combined with radiotherapy and chemotherapy can improve the prognosis of patients with early stage. It′s feasible to have fertility-preserving treatment for specific patients. To improve the clinical management, we review its clinical characteristics and advances in both diagnosis and treatment.