Aggressive angiomyxoma of the female urethra

Aggressive angiomyxoma is a rare locally invasive mesenchymal neoplasm of unknown pathogenesis arising predominantly in pelvic and perineal tissues of adult women. Surgical excision is the classical management but is associated with the risk of recurrence, especially with incomplete excision. There is a proposed role for adjuvant hormonal therapy. We report a very rare case of urethral aggressive angiomyxoma, managed by surgical excision, in a 40-year-old woman who presented with severe hematuria with clot retention.