Extragenital malignant mixed mesodermal müllerian tumors (MT) are rare neoplasms with poor prognosis. Most of them affect women older than 60 years. We present here a case with primary peritoneal malignant mixed müllerian tumor occurring in a young woman who underwent previous hysterectomy and double oophorectomy secondary to a benign disease. We report on the clinical, pathological, and immunohistochemical features of this lesion, which was composed of a poorly differentiated epithelial component and multiple areas of chondromatous differentiation. Along with a brief review of previously reported literature about genital and extragenital MT, some concepts relevant to this case are discussed.
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