A twelve-day old girl was transfered to Sendai Red Cross Hospital with an abdominal tumor. Computer tomography scanning and echogram showed a large irregular solid mass at the presacral region. Exproratory laparotomy revealed a large tumor arising from presacral retroperitoneal region and a specimen was taken. Histopathological, histochemical and ultrastructural examinations identified the diagnosis of malignant schwannoma. Actinomycin D was given in the amount of 10μg/kg intravenously daily for 5 days. The day after initiation of chemotherapy, rapid decrease in white blood cells and platelets resulted bleeding tendency, general convulsion and finally caused a death of this patient. Usefullness of histochemical and electronmicroscopical examinations in the diagnosis for neurogenic tumors is discusseed in relation to this case.