Purpose: Endometrial stromal sarcoma (ESS) is a rare malignancy of the uterus. Most cases are incidentally diagnosed on histological examination of hysterectomy specimens. Evidence on the management is accrued mainly from retrospective studies. This study aims to review the clinical experience of a district hospital in the diagnosis and management of this rare tumour over a 12-year period. Methods: Information on cases of ESS from 1995 to 2007 were retrieved from the histological database. All case files of identified patients were analysed and data extrapolated. A literature review was performed. Results: There were seven cases identified over this time period highlighting the rarity of ESS. Most cases were low-grade ESS and diagnosed retrospectively following surgery for presumed benign pathology. Cases with high-grade ESS and advanced stage low-grade ESS received adjuvant therapy. Conclusion: The primary treatment of ESS is surgery. The role of adjuvant therapy remains debatable, but generally involves radiotherapy, chemotherapy and hormonal therapy Optimal treatment protocols may be achieved by the conduct of randomised controlled clinical trials.