Diffuse Idiopathic Skeletal Hyperostosis (DISH) had been regarded as a radiologic entity with an innocuous clinical course, but recent years it has been reported as potentially devastating, such as dysphagia, fracture following minor trauma and myelopathy. Spondyloepiphyseal dysplasia congenita is a rare condition with spinal involvement such as flat vertebrae and spinal deformities. We experienced a case of DISH with thoracic myelopathy in spondyloepiphyseal dysplasia congenita patient, and this report can elicit further concern to these disease entities.