Tumors with a predominant myxoid stroma are rare in the uterus. When encountered, however, they pose a diagnostic challenge. Traditionally myxoid leiomyosarcoma has been the most important consideration in this category, given its adverse prognosis and deceptively bland morphology. Conventional features of malignancy are variably present; in contrast, an infiltrative tumor border is a consistent pathologic characteristic. More recently, previously under-recognized lesions have been identified, in part due to our growing knowledge of their underlying molecular alterations: uterine inflammatory myofibroblastic tumor frequently harbors ALK rearrangements and a novel ZC3H7B-BCOR gene fusion has been described in a subset of myxoid high-grade endometrial stromal sarcomas. These tumors need to be distinguished from myxoid leiomyosarcoma, as by comparison have a less aggressive course and are amenable to targeted treatments. In addition, uterine mesenchymal tumors with malignant potential need to be distinguished from benign tumors and epithelial and mixed malignancies. This review aims to discuss our current understanding of the most common uterine myxoid neoplasms: their clinical features, their distinguishing histopathologic, immunohistochemical, and molecular features and the clues and pitfalls in their diagnosis.