We describe a case of a concomitant well-differentiated endometrial endomet
rioid adenocarcinoma and leiomyosarcoma of the uterus in a 66-year-old woma
n who presented with a 6-month history of vaginal bleeding. The patient und
erwent total hysterectomy for endometrial carcinoma diagnosed by endometria
l biopsy. Gross examination of the specimen revealed an endometrial mass bu
lging into the endometrial cavity and an underlying well-circumscribed nodu
le separated from the endometrial mass by a myometrial band. Frozen section
performed at the time of the total hysterectomy rendered a diagnosis of ma
lignant mixed-mullerian tumor. Histologic examination of the permanent sect
ions revealed well-differentiated endometrial endometrioid adenocarcinoma c
learly separated from a high-grade leiomyosarcoma. Differential diagnosis i
ncluded malignant mixed-mullerian tumor. However, no admixture of carcinoma
tous and sarcomatous elements was present. There were no heterologous eleme
nts. To the best of our knowledge, no similar case has been described in th
e English literature.