This chapter will cover the pathology of uncommon uterine mesenchymal neoplasms, including inflammatory myofibroblastic tumor, perivascular epithelioid cell tumor (PEComa), uterine tumor resembling ovarian sex cord tumor (UTROSCT), NTRK-rearranged uterine sarcoma, and rhabdomyosarcoma. Several other mesenchymal neoplasms that are exceptionally rare in the uterus, but should always be considered in the differential diagnosis, will be reviewed, along with the spectrum of mixed epithelial-mesenchymal lesions. Discussion will focus upon the salient morphologic, immunohistochemical, and molecular features that help to classify these challenging tumors. In addition, targeted therapy options and syndromic associations will be covered where applicable.