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Uterine sarcomas are rare heterogeneous neoplasms, comprising only a small percentage of uterine malignancies. There is a diverse array of uterine sarcoma sub-types, each with distinct molecular, clinical, and behavioral features. In recent years, there has been significant advancement in all facets of the multi-disciplinary management of uterine sarcomas. Despite this, several challenges regarding optimization of treatment remain. Pre-operative diagnosis can be difficult, as imaging and biopsy may not reproducibly distinguish uterine sarcomas from benign entities. Early-stage uterine sarcomas are potentially curable with surgery; however, there remains a high risk of recurrence, and the use of adjuvant treatment is controversial. There is also a lack of high-quality data to guide best practices for fertility-sparing surgery in younger patients. Optimal management requires multi-disciplinary discussion by clinicians experienced in sarcoma management at specialized sarcoma centers, as initial management may have a profound impact on patient outcomes. In this review, we summarize and address many of these challenges facing patients with early-stage uterine sarcoma, and provide a comprehensive update on the clinical features, the molecular and genomic pathogenesis, and the current diagnostic and treatment landscape for these patients.