Tumor lysis syndrome (TLS) is a well-known oncologic emergency. It is a constellation of metabolic derangements usually observed in hematological malignancies due to rapid cell lysis, typically due to chemotherapy or radiotherapy initiation. Spontaneous TLS is an unusual complication in solid malignancies, and only a few cases have previously been reported for spontaneous TLS in gynecological malignancies. We report a case of TLS in a 50-year-old female patient shortly after resection of high-grade uterine sarcoma. We review previous TLS cases in uterine malignancies and the associated morbidity and mortality.