An 18-year-old male with Maroteaux-Lamy syndrome was presented with spastic quadriparesis. Magnetic resonance imaging of whole spinal canal revealed stenosis at multiple levels of cervical, thoracic and lumbar regions. By the guidance of combined evaluations of neurological examination, neuroradiological and electrophysiological findings, the most responsible spinal segment was detected each time he developed myelopathy and he underwent craniocervical, cervical and thoracolumbar decompressions consecutively. Ligamentum flavum hypertrophy was found to be the principal pathology responsible for the cord compression and myelopathy for all levels. The etiology of myelopathy and priority of the level for which decompression should be done in diffuse spinal stenosis were discussed with the literature review of Maroteaux-Lamy syndrome.