The extraskeletal myxoid chondrosarcoma (CME) is a rare malignant soft tissue tumour described as a distinct clinical, histological, immunohistochemical, genetical and evolutive entity. It represents only 2.5% of soft tissue sarcomas. Its individualization is important because it has a long and indolent clinical course, and tumour-related death often occurs after a long survival period. The diagnostic key is morphological supported by immunohistochemistry and genetics t (9; 22) that allow differentiating it from other tumours with myxoid stroma and from chordoma. This report describes a patient with paravertebral extraskeletal myxoid chondrosrcoma with a high locoregional extension.