We report a 3-year and 11-month-old Caucasian female, who initially presented with an unresectable hepatic angiosarcoma. After three courses of chemotherapy with adriamycin/cisplatin, the tumor decreased in size considerably, allowing complete surgical resection. She also received postoperative chemotherapy with alternating cycles of ifosfamide/etoposide, cisplatinum/adriamycin, and vincristine/actinomycin D/cyclophosphamide for 18 months. She remains disease-free for greater than 44 months from the initial diagnosis. Our experience suggests that total excision of the tumor, together with an aggressive chemotherapy regimen, can improve the disease-free survival for children with this highly malignant vascular tumor of the liver.