Sex cord tumor with annular tubules (SCTAT) is a rare special type of ovarian sex cordstromal tumor, which has not achieved a standard treatment regimen currently. The aim of this report was to provide possible reference for treatment and supervision of recurrent advanced malignant SCTAT. A 33-year-old unmarried female with malignant SCTAT emerged recurrence after the cytoreductive surgery but preserved fertility and postoperative chemotherapy (3 cycles PEB regimens) 12 months. She was performed preoperative adjuvant chemotherapy (3 cycles, paclitaxel liposome and nedaplatin) before the third operation of secondary cytoreductive surgery, followed by platinum-based chemotherapy (7 cycles, TC regimens) timely because of dissatisfactory efficacy under the evaluation in combination with prior comprehensive treatment and PET/CT. The surgical stage was IIIB. She acquired complete remission after therapy and needed to regularly follow up every 3-6 months for 2 years and every 6-12 months after 2 years. There was no evidence of malignant SCTAT recurrence at the follow-up 24 months past the third surgery. No abnormality was found in other examination indexes, and menstruation was regular. The regular supervision of the patient was continually performed. The diagnosis of recurrent advanced malignant SCTAT was established on history and final pathology examination, whose prognosis mainly depended on the appropriate strategy of surgery and chemotherapy regimens. The classical TC regimens could be considered if the efficacy of other chemotherapy regimens were unsatisfactory. Regular follow-up was essential.