Ewing sarcoma (ES) is a high-grade malignancy usually identified in children and adolescents. Primary extraskeletal lesions of ES are rarely detected and no established treatments are available for advanced ES. A fifteen-year-old female presented with abdomen discomfort, and had high titer of neuron specific enolase (NSE), a specific tumor marker for neuroendocrine tumors. Radiological findings showed a large kidney mass with many nodules in bilateral lung and ilium. She was diagnosed as ES after total nephrectomy. She received chemotherapy and autologous peripheral blood stem cell transplantation accompanied with high-dose chemotherapy, but the tumor recurred in lung and bone. Our case suggests that it is necessary to differentiate ES for childhood and adolescent patients of large renal tumor with metastasis having high value of NSE. Developing a new treatment including molecular targeted therapy is warranted for patients with advanced ES.