A 9-year-old boy was admitted to our department with a chief complaint of dyspnea. Emergency thoracotomy revealed a huge hard mass which arised from the right 9th intercostal muscle and occupied the almost all of the right hemithorax. Total resection of the tumor with right 8th, 9th and 10th ribs was performed. 14 cases of rhabdomyosarcoma arising from the chest wall were collected from the Japanese literature. Among them, no long-survivors were found except in the patients with total re-section.
