Aggressive angiomyxoma (AA) is a rare mesenchymal neoplasm of the female pelvis and vulva, which has a tendency for local recurrence. AA is occasionally misdiagnosed as Bartholin's gland cyst or abscess, lipoma, simple labial cyst, or soft tissue tumors. We describe a case of AA on the outside of the left labium majus pudendi in a 28-year-old female. We were able to make the preoperative diagnosis of AA by magnetic resonance imaging (MRI) and ultrasound (US), and to perform complete surgical excision with a transperineal minimally invasive approach. The novelty of this case is the use of ultrasound for the diagnostic workup of the perineal tumor and determination of the incision line.
Keywords: Aggressive angiomyxoma; Perineal tumor; Ultrasound; Vulva.