Aggressive angiomyxoma (AAM) is a rare soft tissue mesenchymal tumor, which mainly occurs in the pelvic cavity and perineum of women of childbearing age. AAM has the characteristics of strong invasion and easy recurrence. This article reports a case of the preoperative imaging manifestations, surgical findings, pathological diagnosis, and postoperative recurrence in the pelvic cavity. The imaging features, differential diagnosis, treatment, and prognosis of AAM were summarized through literature review.