Background: The vast majority of primary vaginal cancers are squamous cell carcinoma. The leiomyosarcoma of the vagina is extremely rare neoplasm. It comprising 2% to 3% of vaginal malignancies. Only 77 cases have been reported up to date in Anglo-Saxon literature .
Aim: Report of a new case
Case: We report a case of a patient 43 years old, single and presented a tumor of 7 cm to the detriment of the posterior wall of the vagina. The treatment consisted of a radical colpohysterectomy followed by postoperative radiotherapy. After 40 months, she is still alive without local or distant recurrence.
Conclusion: This is a rare observation. The surgery is the basic treatment, the poor prognosis of these tumors remains linked especially to the histological grade.