Aggressive angiomyxoma is a rare tumour, which presents as a painless expanding mass in the vulvo-vaginal region. It usually occurs in 2nd to 3rd decades of life. It behaves like a low-grade sarcoma with high propensity for local spread and recurrence and can involve vulva, perineum, vagina and urinary bladder. It is difficult to differentiate clinically this tumour from other mesenchymal tumours occurring in this region. Microscopically it must be differentiated from malignant tumours with myxoid change, like liposarcoma and myxoid leiomyosarcoma. Hence, histopathologic examination has a central role in diagnosis of this tumour. We are presenting a case of young woman, who came with history of swelling in vulva and perineum. Imaging studies in the perineal region revealed a large pelvic mass. Fine needle aspiration cytology was inconclusive due to scanty material. Enucleation of mass was attempted in first surgery but complete extirpation could not be performed. The swelling recurred within few weeks after surgery and required irradiation. A second surgery, however, was successful in complete removal of the tumour.