Aggressive angiomyxoma (AA) is a rare, slow-growing mesenchymal neoplasm of vulvo-perineal region. Although AA is common in females of reproductive age, only a few cases during pregnancy have been documented in the English literature. It carries a high risk of local recurrence but rarely metastasizes. The high recurrence rate can partially be due to inadequate excision, which may be due to an incorrect preoperative diagnosis. We present a case of 25-year-old pregnant female presenting with a painless and soft mass attached to left labia majora by a stalk. This mass was clinically thought to be a lipoma. It was completely excised and was diagnosed as AA on histopathology. Gynecologists should consider the diagnosis of AA when a young female especially during her pregnancy presents with a vulvo-perineal mass. Incorrect diagnosis may lead to incomplete excision and recurrence.
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