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Hereditary multiple exostoses or diaphyseal aclasia (first described by Stanley in 1849) is not a rare disorder. In 1925, the hereditary nature of the disorder was shown conclusively by Stocks and Barrington. Jaffe (1943) simplified the nomenclature of the disease and theorized about its pathogenesis. Spinal cord compression by an exostosis is a rare complication of this disease. We have been able to trace only 19 cases reported so far in the literature (excluding the present one).