Aggressive angiomyxoma is a rare neoplasm that arise from soft tissue of the perineum and usually affects young woman. It has a high risk of local recurrence due to infiltrative lesion. This tumor should be distinguished from benign and malignant tumor or tumor like lesion of the perineum. We experienced a case of an aggressive angiomyxoma on soft tissue of the vaginal wall in 43-year-old woman and reported it with a brief review of literature.