Aggressive angiomyxoma is an uncommon mesenchymal myxoid tumor that is characterized by slow growth and frequent local recurrence. It is currently regarded as a nonmetastasizing tumor. We describe a case of recurrent aggressive angiomyxoma with invasion into the veins including the inferior vena cava and the right atrium and with pulmonary metastases. Our case, together with those unusual cases documented in previous reports, may lead to a reappraisal of the nature of aggressive angiomyxoma.