The current 2014 World Health Organization (WHO) Classification of mixed epithelial and mesenchymal tumours of the uterus includes categories of carcinosarcoma, adenosarcoma, adenofibroma, adenomyoma and atypical polypoid adenomyoma, the last two lesions being composed of an admixture of benign epithelial and mesenchymal elements with a prominent smooth muscle component. In this review, each of these categories of uterine neoplasm is covered with an emphasis on practical tips for the surgical pathologist and new developments. In particular, helpful clues in the distinction between carcinosarcoma and dedifferentiated endometrial carcinoma will be discussed. In addition, salient features to help distinguish between adenofibroma, adenosarcoma, embryonal rhabdomyosarcoma and other mesenchymal neoplasms in the differential diagnosis will be outlined. Finally, a discussion of adenomyoma and its main differential diagnostic considerations will be covered.