Reported in this paper are two cases of aggressive perineovulvar angiomyxoma. Soft, painless tumours with gelatinous cut surface were detected in either patient, two women aged 32 and 46 years. Histological findings included fibromyxoid stroma with spindle-shaped and stellate cells as well as vascularization in striking abundance. Most of the vessels were thin-walled. There was no plexiform arborization. Strongly atypical nuclei or mitoses or necrotic foci were not recordable. The tumour exhibited infiltrative growth. Tumour cells could be immunohistochemically associated with antibodies against vimentin, whereas negative responses were recorded from antibodies against S-100 protein, factor-VIII-associated protein and pancytokeratin. Aggressive angiomyxoma is a biologically benign neoplasia prone to recurrence and typically localized in soft tissue of the pelvic region. Metastases so far have never been found. Wide local excision has proved to be the optional therapeutic approach.