Abstract: Objective: To explore the clinicopathological characteristics,the diagnosis and differential diagnosis of aggressive angiomyxoma(AAM).Methods: One case with AAM of pelvic cavity was detected by light microscopy and immunohistochemistry,and the literatures about AAM were reviewed.Results: The high expressions of vimentin,desmin and CD34,and partial expression of SMA were detected by immunohistochemistry in tumor tissue of AAM.Conclusions: The clinical symptoms of AAM are concealed.It is difficult to achieve completely resection by surgery and postoperative recurrence is common.The correct diagnosis can be decided by its pathological characteristics and immunophenotype.