Uterine sarcomas are very rare and diverse. Due to their rarity, pathological diagnosis, surgical treatment, and cure are difficult. They are medically and biologically heterogeneous diseases that are difficult to treat at advanced stages. Recent advances in understanding uterine sarcoma biology have improved diagnostic and clinical management. Promising strategies for treating patients with uterine leiomyosarcoma involve targeting the DNA damage repair pathways and suppressing the activity of the macrophage immune system. Endometrial stromal sarcomas often exhibit mutations in the Wnt, cyclin D-CDK4/6-Rb, and MDM2-p53 pathways. Translating these molecular discoveries into new clinical trials to benefit patients with these conditions is crucial.