Objective：To study the clinicopathological characteristics，immunopheno-types，diagnosis and differential diagnosis of vagi－nal endometrial stromal sarcoma. Methods：A retrospective analysis of one case of low grade endometrial stromal sarcoma in the vagi－na was conducted and，was followed up 5 years later with the clinical pathological features of local recurrence cases combing with the review of literature at home and abroad. Results：The histological morphology and immunohistochemistry of local recurrence after 5 years of the low level endometrial sarcoma of the vagina were consistent with 5 years ago. There are few cases of endometrial sarcoma in the vagina，and 14 cases have been reported in both Chinese and English. So far，no local recurrence case has been reported. Conclusion：Although the primary vaginal endometrial stromal sarcoma is low grade endometrial stromal sarcoma（LGESS），the tumor may still relapse after a few years of resection，and it is suggested that patients with this disease should be followed up for a long time.