BACKGROUND: Aggressive angiomyxoma is a type of mesenchymal tumor occurring predominantly in the pelvic and perineal region. The aim of our study was to reveal our experience with gonadotropin-releasing hormone (GnRH) treatment in patients with angiomyxoma and provide a comprehensive review of management. PATIENTS AND METHODS: This study is a case-series including seven female patients diagnosed with aggressive angiomyxoma from a single institution, between 2012 and 2020. Follow-up after surgery was ranged between 2-45 months with an average of 17.6 months. Resection was performed in all patients without any complications, and five had received GnRH analogue (Goserelin acetate) therapy after surgery. Immunohistochemistry analyses showed positivity for smooth muscle actin and desmin in all cases, while both estrogen receptor (ER) and progesterone receptor (PR) positivity were identified in 6 patients. None of the seven patients had recurrence during follow up period. CONCLUSION: The mean treatment of aggressive angiomyxoma is surgery, and the use of GnHR analogues in cases with positive ER and PR may be effective in preventing recurrence.