A 46-year-old female patient suffered from uterine malignant leiomyosarcoma. Ten months after abdominal subtotal hysterectomy, multiple lung, bone and spine metastases developed. She received 3 cycles of infusional ADI chemotherapy (adriamycin 60 mg/m2, 96-hour i.v. infusion, dacarbazine 500 mg/m2, 96-hour i.v. infusion and ifosfamide 5,000 mg/m2, 72-hour i.v. infusion, repeated every 3 weeks), but the metastatic tumors progressed. An empirical ADI-TAM chemotherapy which incorporated high-dose tamoxifen (TAM) (150 mg/m2/day, in 4 divided doses) into the original ADI regimen was applied from the fourth cycle. High-dose tamoxifen consisted of 240 mg/day for 4 days during chemotherapy and 40 mg/day between cycles. Good partial response with nearly total tumor reduction was achieved after 3 cycles of ADI-TAM therapy. The excellent tumor response by simple addition of high-dose tamoxifen into a previously inactive ADI regimen advocates further investigation for the development of chemotherapy in metastatic uterine leiomyosarcoma.