Malignant mesothelioma is a very rare tumor in childhood. Presently, treatment of this disease continues to be frustrating and prognosis remains poor. We here report a pediatric case of malignant pleural mesothelioma who gave a complete response to ICE-VAC chemotherapy regimen and achieved a long-term survival. An eight-year-old girl underwent exploratory thoracotomy and decortication because of a unilateral loculated and multicystic pleural effusion. Histopathological diagnosis was sarcomatoid pleural malignant mesothelioma. After decortication, chemotherapy with ICE (ifosfamide, carboplatin, etoposide) - VAC (vincristine, adriamycin, cyclophosphamide) combination was started. Six courses of chemotherapy resulted in complete clinical and radiological tumor response. She did not receive any further therapy and remains disease-free three years after the first remission. ICE-VAC chemotherapy combination resulted in a complete tumor response and a long-term disease-free survival for the presented case. The efficacy of this chemotherapy regimen in malignant mesothelioma needs to be documented in future trials.