Aggressive angiomyxoma is a rare mesenchymal tumor mostly observed in women of childbearing age. Although it is often lo- cated in the vulva, it can also be observed in the paravaginal, perirectal areas, perineum and pelvis. It appears as a locally aggressive, large, in- filtrative mass. Complete surgical excision is often difficult and has high recurrence rates. Magnetic resonance imaging (MRI) plays an important role in preoperative diagnosis of aggressive angiomyxoma. The typical swirling appearance on T2-weighted and post-contrast T1-weighted MRI series is diagnostic for aggressive angiomyxoma. Here, we present a 51-year-old female patient with pelvic-perineal aggressive angiomyxoma, accompanied by radiological and pathological findings.