Abstract: Aggressive angiomyxoma (AA) is a rare mesenchymal tumor that generally occurs in the perineum and pelvis of women of reproductive age. Despite its characteristics of local infiltration and recurrence, it is not considered as a malignant tumor. The third (2003) and fourth (2013) editions of the World Health Organization Classification of Bone and Soft Tissue tumors classified it as a tumor of uncertain differentiation and named it "aggressive (deep) angiomyxoma." So far, more than 350 cases of aggressive angiomyxoma have been reported worldwide, mostly in the form of case reports and literature reviews. In this review, we present updated knowledge on AA.