Aggressive angiomyxoma (AAM) is a rare locally aggressive neoplasm of mesenchymal origin which occurs mainly in adult women. High local recurrence rate after surgery is due to incomplete surgical excision and most of the reported cases originate in the pelvis. We present a case of massive angiomyxoma arising from the abdomen and extending into the pelvis and perineum. A 23-year-old woman presented with a swelling of the left lower abdomen with discomfort and pain for 4 years. The swelling increased in size progressively and involved the perineum. At surgery the mass was abutting the urinary bladder and rectum. On microscopy there were typical features of AAM. Our case is being reported due to the rarity of its presentation. Close follow-up is necessary as there is high chance of recurrence.