Abstract: Objective: To investigate the clinical features, management, and prognosis of endometrium clear cellcarcinoma. Methods: A total of 48 cases of endometrium clear cell carcinoma and 601 cases of endometrioid adenocarci-noma seen in our hospital from January 1997 to December 2006 were analyzed retrospectively. Results: The mean age ofthe patients with endometrium clear cell carcinoma and patients with endometrioid adenocarcinoma was 54.1 years and51.7 years, respectively, with a significant difference (P<0.05). Compared with the endometrioid adenocarcinoma, en-dometrium clear cell carcinoma presented with higher metastasis rate of pelvis lymphoid node and vessel and infiltrate rateof muscular layer (P<0.05). These 48 cases of endometrium clear cell carcinoma all underwent surgery. After surgery, 14patients had chemotherapy, 13 patients had radiation therapy (RT), two patients had hormone therapy, six patients had acombination of RT and chemotherapy, one patient had a combination of chemotherapy and hormone therapy, two patientshad a combination of RT and hormone therapy, and 10 patients didn't receive any adjuvant therapies. Conclusion: En-dometrium clear cell carcinoma comprises a small percentage of endometrial cancers. The onset age is higher than that ofendometrioid adenocarcinoma. It has been shown to be associated with high metastasis rate and poor survival. Patientswith endometrium clear cell carcinoma should be treated with complete staging surgery and combination of regional RTand chemotherapy.