Aggressive angiomyxoma (AA) is an uncommon mesenchymal tumor that mainly affects the perineum and pelvis in females of reproductive age. AA is commonly misdiagnosed as a Bartholin's duct cyst, abscess or levator hernia. A 42-year-old female presented with a large, progressive and painless perineal swelling. She was initially diagnosed with an obturator hernia three times over the past 10 years and underwent multiple surgeries for recurrences. For this presentation, she underwent exploratory laparotomy. No hernia was identified. A large mass over the perineal area extending from the vulvar commissure to the anus was visualized. The diagnosis of AA was made on histopathology. Leuprolide (GnRHa) was used as hormonal therapy postoperatively. AA should be considered in any pelvic mass in a young female that recurs after excision. Prompt diagnosis will contribute toward minimizing local destruction of surrounding structures.
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