Extraskeletal myxoid chondrosarcoma is a rare soft tissue sarcoma. Surgery is the cornerstone of the management of this tumour. The response rate to chemotherapy has been very poor; with the exception of one reported case which showed promising results, overall results are disappointing because no significant radiologic or clinical responses have been noted with chemotherapy. Here we report the case of a 15-year-old girl who presented with extraskeletal myxoid chondrosarcoma in the sacrococcygeal region which was regarded as unresectable. After four cycles of chemotherapy the mass showed complete remission which has lasted >6 months.