Introduction: Transitional cell carcinoma (TCC) of the ovary resembles urothelial rather than ovarian surface epithelium. The histopathology pattern is diagnostic. Ovary TCC typically shows undulating, diffuse, insular, and trabecular growth patterns. Case presentation: A 72-year-old postmenopausal woman presented with progressive enlargement of abdominal girth and diffuse pain in October 2008. Abdominal ultrasound showed a pelvic mass with homogeneous echogenicity. Routine biologic test results were all within normal ranges; CA-125 below upper limit (35 mUI/l). Surgical staging procedures including total abdominal hysterectomy, bilateral salpingo-oophorectomy, infracolic omentectomy, and pelvic lymph node dissection were performed. The final diagnosis was TCC of the ovary, grade 3, FIGO stage IIIC. The patient underwent six cycles of chemotherapy with carboplatin and paclitaxel. She was disease-free for 10 months. At progression, liposomal doxorubicin was initiated. At new progression, reintroduction with carboplatin and paclitaxel plus bevacizumab was administered. She had complete response documented by positron emission tomography-computed tomography (PET-CT) scan. Conclusion: Primary TCC of the ovary is a rare subtype of epithelial ovarian cancer. Surgical resection remains the primary therapeutic approach, followed in some cases by adjuvant chemotherapy. It is sensitive to platinum-based combinations. The prognosis of these tumors is better than for other types of ovarian cancers.