The histogenesis of aggressive angiomyxoma, an uncommon mesenchymal neoplasm of the vulvar, pelvic, and perineal soft tissues, is poorly understood, although the neoplastic cells exhibit a myofibroblastic phenotype. Cytogenetic studies of aggressive angiomyxoma are scarce; however, a nonrandom involvement of the 12q15 region where the high mobility group (HMG) protein HMGA2, an architectural transcription factor expressed primarily during embryogenesis, is located has been suggested. HMGA2 involvement has also been described in a variety of benign gynecologic mesenchymal neoplasms. We report an additional case of HMGA2 rearrangement in vulvar aggressive angiomyxoma, using fluorescence in situ hybridization.