A 24-year-old woman was admitted due to dyspnea on exertion. A chest roentgenogram revealed a massive tumor originating from the right anterior chest wall when pleural effusion was drained. Diagnosis of Askin tumor was made based on light microscopic findings characterized by composing with small round cells, immunocytochemical findings suggestive of neuroectodermal origin, and cytogenetic analysis demonstrating the chromosomal translocation (11; 22). After intensive combined modality therapy, including chemotherapy, irradiation, and additional surgery, she was followed up as an outpatient and has remained disease-free for 16 months after initial diagnosis.