We describe an extremely rare case of advanced pure primary ovarian squamous cell carcinoma (SCC), treated by adjuvant chemotherapy with dose-dense paclitaxel combined with carboplatin (dd-TC) plus the combination chemotherapy with irinotecan and cisplatin (CPT-P), with long-term recurrence-free survival. A 71-year-old woman complaining of lower abdominal pain was referred to our hospital and a 7-cm-diameter solid tumor was identified. She was diagnosed with a left ovarian tumor that was highly suspicious for malignancy based on ultrasonography, magnetic resonance imaging, and contrast-enhanced computed tomography. Bilateral salpingo-oophorectomy, low-anterior colon resection, and colostomy were performed. Intra- and post-operative histopathological diagnosis revealed International Federation of Gynecology and Obstetrics stage IIIc well-differentiated pure ovarian SCC. As adjuvant chemotherapy, 2 courses of dd-TC were administered, followed by 3 courses of CPT-P; the patient then underwent 4 additional courses of dd-TC. Both regimens were effective and there has been no recurrence or metastasis thus far in the 5 years since the operation.

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