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Angiomyxoma occurs mostly in 30- to 40-year-old females and is described histologically as a mesenchymal tumor, composed of fibroblasts within a strong myxoid background. It occurs mainly in the female pelvis, vulva or perineum, and grows slowly. Treatment is surgical excision. Unfortunately, there is a relatively high rate of recurrence because the exact extent of the tumor is difficult for the surgeon to determine. We report a case of aggressive angiomyxoma combined with uterine myoma, and discuss the characteristics of its images. Surgical excision of the tumor was performed, and adjuvant treatment was given for local recurrence.