Aggressive angiomyxoma is a rare mesenchymal tumor with a locally infiltrative growth pattern. It preferentially involves the pelvic and perennial regions. It is a locally infiltrative neoplasm with a marked tendency to recurrence, although distant metastases are extremely rare. Radical surgical resection is considered the mainstay of treatment. However, the risk of recurrence is high, so therefore multimodal treatment is needed. Knowing that most of these tumors are estrogen and progesterone receptor positive, it has been hypothesized that they may respond to hormone treatment. We report the case of a 34-year-old woman with a tumor in right gluteal region. The biopsy revealed aggressive angiomyxoma. The patient was treated with surgery. However, 3 months later she developed locoregional recurrence. We started systemic hormonal treatment with tamoxifen and goserelin. After 14 months of treatment there was partial response.