A 39-year-old woman having a pure gonadal choriocarcinoma with lung metastasis was referred to our hospital after hysterectomy and bilateral salpingo-oophorectomy. She was found to have a 45XO/46XY karyotype and gonadal dysgenesis. The patient's serum beta-hCG was normalized after six courses of chemotherapy with cisplatin and etoposide of conventional dose (100 mg/m2, 100 mg/m2 x 3 days), but began to fluctuate. Thoracoscopic resection of a remaining pleural lesion was negative for malignancy. However, the disease relapsed as multiple metastatic nodules in bilateral lung fields and the mediastinum. After one course of priming chemotherapy with conventional dose (1 g/m2 cyclophosphamide, 400 mg/m2 carboplatin, and 500 mg/m2 etoposide), high-dose chemotherapy with a total dose of 1500 mg/m2 carboplatin, 1200 mg/m2 etoposide, and 5 g/m2 ifosfamide followed by autologous bone marrow transplantation and peripheral stem cell support was given. Thoracoscopic surgery was performed to resect two residual solitary metastatic lung lesions. With these salvage treatments, the patient obtained complete remission and remained disease free at last follow-up (17 months). Our result suggests that high-dose chemotherapy may be effective in chemosensitive nongestational choriocarcinoma when first chemotherapy has failed.