Simple SummaryDeep (aggressive) angiomyxoma (DAM) is an extremely rare primary neoplasm of the vulva whose growth is particularly favored during pregnancy. To date, neither the origin nor the optimal management of DAM are clear, especially in pregnancy. Therefore, we present here a systematic review that could be a useful tool to provide further knowledge about the behavior of and the management strategies for this rare malignancy.AbstractDeep (aggressive) angiomyxoma of the lower genital tract is a rare malignancy affecting women of reproductive age. Being a hormone-sensitive tumor, its growth is particularly benefitted during pregnancy. Surgical excision with complete resection is indicated, even if a wait-and-see approach can be considered until delivery, to avoid destructive surgeries. The mode of delivery is to be evaluated based on the location and size of the neoplasm; vaginal delivery is not contraindicated, as long as the tumor does not obstruct the birth canal. Positive surgical margins are the most important prognostic factor for recurrence. Adjuvant therapy with gonadotropin-releasing hormone analogues may be proposed after pregnancy, in the case of non-radical surgery. Despite the high local relapse rate, the outcomes for mother and child are favorable. Since recurrences can occur after many years, the patient should be included in long-term follow-up.