A case of aggressive angiomyxoma in a 38-year-old pregnant woman is described. These clinicopathologically distinctive soft tissue tumors comprise a quite rare entity. They are usually located in the pelvic or perineal soft parts and occur predominantly in women. On gross inspection, these lesions are typically soft bulky masses, frequently with a multilobulated contour and of gelatinous appearance. They are characterized by slow growth, infiltration of adjacent structures, absence of distant metastases and a tendency to recur locally, probably secondary to incomplete excision. Therefore, treatment should consist of wide surgical excision, as complete as technically possible. This is the second case of aggressive angiomyxoma of the pelvis occurring in a pregnant woman.