Aggressive angiomyxoma (AA) was identified in 1983 and 250 cases of this rare tumor have since been reported in the literature. It is characterized by a locally infiltrative and recurrent nature; however, it rarely shows distant metastasis. Surgical managements can successfully treat AA patients but may result in a significant morbidity due to the large size and infiltration of the tumor. Less radical surgeries have recently been recommended in the treatment of this tumor, but adjuvant therapies have not yet been fully established. The authors report here two AA cases that were treated at their hospital, with a brief review of the literature.