Abstract A case of extragential malignant mixed mullerian tumor (MMMT) of the heterologous type 11 years after treatment of a Stage I ovarian neoplasm in a 65-year-old patient is described. There was no evidence of a histologic relationship between these two neoplasms. This case represents the 10th report of an extragenital MMMT. The medical literature and histogenesis of this rare tumor are discussed.