Abstract
Aggressive angiomyxoma (AA) is a rare benign soft tissue tumor seen in females of the reproductive age group and involving the lower pelvis and perineal soft tissue. It is characterized by the presence of both myxoid and vascular components. We present a case of a 36-year-old female who presented with right buttock swelling. Magnetic resonance imaging (MRI) revealed a large perineal swelling suspected to be a giant AA, which was demonstrating hypocellular stroma with a myxoid background in biopsy. AA should be suspected in a female patient presented with large perineal swelling. MRI should be the investigation of choice for radiological diagnosis of this rare entity to be confirmed by a pathology report.