Introduction:
Extragenital malignant mixed Müllerian tumor is an extremely rare presentation of malignant mixed Müllerian tumor, especially when combined with a synchronous ovarian cancer.
Case presentation:
We report the clinical course and pathologic findings of a case of mesorectal malignant mixed Müllerian tumor with synchronous ovarian cancer, in a 50-year-old, gravida 0, para 0, Han Chinese woman with regular menstruation. This is the sixteenth case in the English literature of extragenital malignant mixed Müllerian tumor combined with synchronous or metachronous malignancy reported.
Conclusion:
Although extragenital malignant mixed Müllerian tumor is very rare and has a poor prognososis, a longer survival time might be achieved with treatment by cytoreductive surgery, radiotherapy and chemotherapy.