Clear cell endometrial cancer (CCE) is an uncommon but important disease because of its aggressive behavior. Furthermore, prospective, randomized studies are either too difficult or impossible because of the small number of women affected. This review explores the differences between clear cell and endometrioid endometrial cancer. In addition, it uses available evidence to determine the best approach to management. Medline was searched between January 1, 1966 and December 31, 2008 for all publications in English where the studied population included women diagnosed with CCE. Qualifying studies must have had at least 30 patients. Clear cell histology is diagnosed in less than 6% of all endometrial cancers and its incidence increases with age. Diagnosis can be made using the same tests that are used in the diagnosis of other types of endometrial cancer. Clear cell histology is morphologically and genetically different from the more prevalent endometrioid endometrial cancer histology. It shares many similarities with clear cell neoplasms of the ovary and kidney. Comprehensive surgical staging is critical in order to plan appropriate postoperative management. Adjuvant pelvic and/or whole abdominal radiotherapy have not been shown to be clearly beneficial in women diagnosed with clear cell endometrial cancer. Adjuvant chemotherapy with cisplatinum, taxol and doxorubicin either in a doublet or triplet combination has demonstrated efficacy. Women diagnosed with CCE require comprehensive surgical staging. Platinum based adjuvant chemotherapy in a doublet or triplet format in combination with paclitaxel and/or doxorubicin should be considered as part of treatment of these women. Careful long term surveillance following treatment is indicated given the higher rate of recurrence compared to endometrioid endometrial cancer.