SMARACA4 deficient uterine sarcoma (SDUS) is a new type of cervical sarcoma discovered in recent years, with high invasion, poor prognosis and very rare occurrence. Case: This article shares a 53 years old woman with postmenopausal vaginal bleeding. Colposcopy examination and cervical biopsy confirmed SMARACA4 Deficient Uterine Sarcoma. She underwent total hysterectomy and bilateral salpingo-oophorectomy, and the postoperative examination revealed that the patient also had endometrial cancer. She was treated with supplementary radiotherapy and chemotherapy and passed away 7 months after surgery. Conclusion: SMARACA4 Deficient Uterine Sarcoma still has poor prognosis after active surgical treatment and supplementary treatment. During surgery do not removed pelvic lymph nodes did not shorten the patient’s survival period. Early diagnosis and identification of high-risk populations are particularly important, and immunotherapy and targeted therapy are still being studied and explored.