Seventeen myelodysplastic patients with progressive extremity spasticity and scoliosis underwent radiological evaluation and surgical treatment. All but one were under 18 years of age at the time of surgical treatment. Duration of the clinical presentation ranged from 1 1/2 to 7 years. Metrizamide was instilled into the subarachnoid space in 12 patients, the lateral ventricle in two, and the hydromyelic cavity in three. Sequential computerized tomograpy scanning after intrathecal instillation of the contrast material clearly demonstrated hydromyelia in nine patients and compression of the brain stem in five. Posterior fossa decompression with plugging of the obex was performed in 12 patients, posterior fossa decompression alone in three, and ventriculoperitoneal (VP) shunting procedures in two. Of the 12 patients who underwent the obex plugging procedures, eight have shown partial or complete resolution of spasticity and an increase in motor strength with no significant postoperative complications. In contrast, posterior fossa decompression or VP shunting procedures alone have not led to a favorable neurological outcome. Hydromyelia may occur more commonly among myelodysplastic patients than previously recognized and may be treated most effectively by the obex plugging procedure.