Objective: To study the clinical pathomorphology of myelodysplasia and research its relationship with the neurological defect of myelodysplasia. Methods: 184 patients with myelodysplasia were diagnosed and evaluated depending on patient history, physical examination, imaging, voiding cystourethrogram and urodynamic tests. 182 patients underwent surgical management. The intraspinal anomalies and their relationships with the spinal cords were observed and recorded during operations. The pathological tissues which were resected during the operations were examined postoperatively. Results: Myelodysplasia was divided into 6 types depending on the intraspinal pathomorphology: type I (tethered spinal cord with morbid terminal filum), type II (tethered spinal cord with fibrous adhesion), type III (lipoma on the spinal cord), type IV (intraspinal cyst), type V (split cord malformations), and type VI (defect of spinal cords and/or nerve roots). Conclusions: Theneurological defect of myelodysplasia results from its intraspinal anomaly and the morphological change of the spinal cord. It should be actively diagnosed and surgically treated to improve the prognosis.