Mesenchymal chondrosarcomas are rare malignant tumors of the bone and soft tissue. Extraskeletal mesenchymal chondrosarcomas, especially those that arise in the central nervous system, are even rarer. Most of those described were intracranially located, with only a very few cases having been reported in an intraspinal region. Therapeutic experience with primary spinal mesenchymal chondrosarcomas is also extremely limited. We report on a case of a 21-year-old man with back pain and bilateral progressive weakness and numbness of the lower extremities. A T1-weighted magnetic resonance image revealed a hypointense tumor located at the T8 level. The tumor was completely excised through a posterior approach. Microscopic examination and immunohistochemical studies confirmed the diagnosis of mesenchymal chondrosarcoma. Spinal irradiation and chemotherapy were also administered for prevention of local recurrence and metastasis. The patient has been symptom-free for 1 year after surgery. Herein, we review the 22 cases of primary intraspinal mesenchymal chondrosarcomas in the literature and discuss their clinical presentations, pathology, imaging studies, treatments, and outcomes.