A 2-month-old boy presented with a huge choroid plexus carcinoma in the left cerebral hemisphere. Almost total resection of the tumor was achieved, but a remnant abruptly enlarged 5 months later, requiring reoperation. After radical resection of the tumor, intraoperative radiation (10 Gy) was delivered. Postoperative chemotherapy was performed with methotrexate and vincristine, selected on the basis of in vitro chemosensitivity testing. No recurrence of the tumor was detected 18 months after the second operation. Radical surgery with these adjuvant therapies may be a good method to control this aggressive disease in infants.