Aggressive angiomyxoma of the vulva presenting as a pedunculated swelling

Aggressive angiomyxoma is a rare, locally aggressive soft tissue tumor that has high propensity for local recurrence. It involves mainly the pelvis, vulva, perineum, vagina and urinary bladder in adult women in the reproductive age. Considering its locally aggressive nature, appropriate management and long-term follow-up is necessary. We describe a case of a 22-year-old young pregnant patient presenting with a large pedunculated swelling on the left labia majora.