Infantile choriocarcinoma of the liver is an extremely rare entity, and outcome has been fatal in almost all published cases. To the authors' knowledge, this is the first report on successful treatment with preoperative chemotherapy. A 10-week-old girl presented with a large liver tumor, ovarian cysts, cardiac insufficiency, progressive hemolytic anemia, and thrombocytopenia. Ultrasound scan and magnetic resonance tomography (MRT) showed the typical pattern of infantile hemangioendothelioma. An emergency laparotomy was performed because of increasing cardiac insufficiency with ligation of the right hepatic artery, tumor biopsy, and subtotal resection of the ovarian cysts. Histology findings showed a choriocarcinoma of the liver and corpus luteum cysts of the ovaries. Serum beta-human chorionic gonadotropin (beta-HCG) was elevated to 1.600.00 U/L. Chemotherapy was initiated with etoposide and cisplatin. When x-ray examination showed development of lung metastases, chemotherapy was intensified with etoposide, cisplatin, and ifosfamid according to the German Study Group of Extracranial Nontesticular Malignant Germ Cell Tumors in Childhood and Adolescence (MAKEI-96). Serum beta-HCG levels decreased further, ultrasound examination showed significant tumor reduction, and pulmonal metastasis could no longer be found in chest x-rays. After the fourth course, a complete tumor resection was achieved by an extended right hemihepatectomy with adjuvant chemotherapy being administered after the operation. The child has been in complete remission for 22 months. The authors' experience shows that chemotherapy is effective for preoperative tumor reduction.