Mesenchymal chondrosarcoma presents multiple challenges. Diagnostic pitfalls include inadequate biopsy samples, which may result in sample error. Sox9 has been proposed as a unique marker for mesenchymal chondrosarcoma which may improve diagnostic specificity. Treatment and prognosis vary considerably. Patients who receive surgery and chemotherapy seem to fare better. Multicenter studies with higher sample numbers may improve our understanding of this malignancy.