A multiparous woman presented with a large bulge protruding from the posterior wall of the vagina, initially diagnosed as a rectocele. Imaging revealed a cystic lesion measuring 9 cm × 6 cm × 3.5 cm suspected to be a developmental cyst. The lesion underwent surgical excision with significant improvement in symptoms. Histological analysis reported an aggressive angiomyxoma (AAM); postoperative hormonal therapies were considered and rigorous follow-up initiated. The authors discuss challenges in investigating and managing AAM with proposals made for improving preoperative diagnosis and optimising postoperative management. Correlating tumour size with symptoms, judicious planning of surgery, rigorous postoperative follow-up and involving the patient in all aspects of decision-making will guide clinicians in providing the best care in the rare entity of AAM.