OBJECTIVE: To evaluate the effects and the toxicity of the protocol of CDV combined with CiE as pre-operative chemotherapy in childhood stage IV neuroblastoma. METHODS: The clinical data of 27 children aged from 1.2 to 8 years with neuroblastoma in stage IV was retrospectively studied. The primary sites of the diseases were abdomen (n=21), posterior mediastinum (n=4) and pelvic cavity (n=2). Twenty-three patients had bone marrow metastasis. Twelve patients had bone metastasis. All patients were treated with the CDV protocol (cyclophosphamide+doxorubicin+vincristine) for 3 cycles and the CiE protocol (cisplatin+etoposide) for 2 cycles. Neuroblastoma therapeutic response evaluation criterion and common terminology criteria for adverse events of National Cancer Institute were used to evaluate effects and chemotherapy-related toxicity. RESULTS: All patients received the pre-operative chemotherapy. The overall response rate was 82%. After chemotherapy, 24 patients received operations. Total resection of primary tumor was found in 14 patients (58%) and part resection in 10 patients (42%). The most common chemotherapy-related toxicity was bone marrow suppression: grade IV suppression of neutrophils (n=27), reduction in hemoglobin (III grade, n=7; IV grade, n=20) and reduction in platelet (III grade, n=2; IV grade, n=25). Infection was found in all patients and was controlled with antibiotics.I or II grade lesions of digestive, liver and kidney were found and could be recovered after therapy. Grade I neurotoxicity occurred in 2 patients (7%). The heart function damage was not found in any of patients. CONCLUSIONS: The protocol of CDV combined with CiE as pre-operative chemotherapy might be effective in children with stage IVneuroblastoma.［Chin J Contemp Pediatr, 2009, 11 (11):885-887］