We report a case of primary transitional cell carcinoma of the endometrium and review the literature which reveals only 12 cases described to date. The patient presented with postmenopausal bleeding, and there were no distinctive clinical features. An endometrial sample taken at hysteroscopy was reported as a poorly differentiated, papillary, nonserous carcinoma with probable squamous metaplasia. The tumor removed at hysterectomy and bilateral salpingo-oophorectomy was almost all transitional cell with small areas of endometrioid, glandular differentiation. Immunohistochemistry demonstrated a similar immunoprofile to endometrioid carcinoma of the endometrium and different from reported cases of transitional cell carcinoma of the ovary and of the urinary tract.