The authors report a case of alveolar rhabdomyosarcoma (RMS) of the thigh complicated by two successive distant relapses shortly after radio- and chemotherapy, treated with etoposide, and resulting in complete long-lasting remission. The schedule of etoposide was 100 mg/ m2/d intravenously for three days weekly for 3 weeks, with an interval of 1 week between courses. This was administered for 11 months. The child is alive without disease and off therapy 20 months after completion of etoposide treatment. Preclinical studies and experience in adults have suggested that the cytotoxic effects of etoposide show a marked dependence on schedule. A divided dose regimen of single-agent etoposide has previously been shown to have activity in relapsed rhabdomyosarcoma, but the outcome or the duration of complete response has not yet been fully evaluated. In out poor-prognosis case, the prolonged administration of etoposide achieved a long-lasting complete response.