Mesenchymal chondrosarcoma is very rare and mostly occurs in lower extremity, especially femur, head and face, and pelvis in order. An eleven-year old male patient suffered from left heel pain for several months and underwent biopsy at another hospital. A Malignant bone tumor was suspected, so the patient was transferred to our hospital. We reviewed the outside pathologic slide, which revealed the diagnosis of mesenchymal chondrosarcoma. It was treated by a below knee amputation. Three months later, a chest computed tomography (CT) revealed multiple metastatic nodules in both lungs. All metastatic nodules were surgically excised and the patient received postoperative chemotherapy. As far as our knowledge, mesenchymal chondrosarcoma in the calcaneus is the first case in the world. Thus, we report a case of mesenchymal chondrosarcoma in the calcaneus with thorough review of documents.