Background: We report a case of a 7-year-old white female who presented with acute, progressive bilateral lower extremity weakness over 48 h. Methods: Case report and presentation of clinical, radiological and pathological data on a single case of chondromyxoid fibroma (CMF) of the T2 vertebral body. Results: Magnetic resonance imaging of the thoracic spine revealed an extensive mass invading the lamina of the second thoracic vertebra, causing extensive cord compression and progressive neurological deterioration. Surgical resection and pathologic study of the mass revealed a CMF. Conclusions: A thorough Medline search has revealed that only 25 cases of spinal CMF have been reported, making this lesion an extremely rare bone tumor.

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