We report a unique case of primary myxoid chondrosarcoma in the posterior mediastinum. This lesion clinically mimicked a neurogenic tumor due to its location and dumbbell appearance. The histogenesis of this tumor is discussed and a skeletal origin from the thoracic vertebrae is suggested. It is concluded that myxoid chondrosarcoma has to be distinguished for the differential diagnosis among the long list of myxoid tumors arising in the posterior mediastinum.