Diffuse idiopathic skeletal hyperostosis (DISH) is a disease affecting mainly the elderly population. With mostly incidental findings, DISH was thought to be a relatively benign disorder affecting the spinal column. However, acute C-spine myelopathy and dysphagia caused by DISH have been reported in the literature. Here, we reported a rare case of acute T10 myelopathy with the presentation of acute cauda equina syndrome associated with DISH. Prompt surgical relief of the ossified ligamentum flavum over the T10 level had resulted in full recovery of the patient. A detailed review of the literature shows that that DISH is neither a clinical infrequent entity, nor an exclusively benign clinical condition. Cares and discretions must be used when treating DISH-associated illness.