Tethered cord syndrome is a progressive disease with a typically insidious onset in infants and children, and which can lead to persistent progress of neurological deficits and a high rate of disability without timely intervention. The purpose of this study was to investigate the curative effect of microsurgery in children with different types of tethered cord syndrome. In this study, we analyzed 326 patients with tethered cord syndrome, aged from 2 months to 14 years old, who were followed for 3–36 months after microscopic surgery. Based on clinical manifestations and imaging findings, these patients were classified into five types: tight filum terminale (53 cases), lipomyelomeningocele (55 cases), lipomatous malformation (124 cases), postoperative adhesions (56 cases), and split cord malformation (38 cases). All patients underwent microsurgery. Curative effects were measured before and 3 months after surgery by Spina Bifida Neurological Scale based on sensory and motor functions, reflexes, and bladder and bowel function. The results showed that Spina Bifida Neurological Scale scores improved in all five types after surgery. Overall effective rates in these patients were 75%. Effective rates were 91% in tight filum terminale, 84% in lipomyelomeningocele, 65% in lipomatous malformation, 75% in postoperative adhesion, and 79% in split cord malformation. Binary logistic regression analysis revealed that types of tethered cord syndrome (lipoma-type or not) and symptom duration before surgery were independent influencing factors of surgical outcome. These results show that therapeutic effect is markedly different in patients with different types of tethered cord syndrome. Suitable clinical classification for tethered cord syndrome will be helpful in predicting prognosis and guiding treatment. This trial has been registered in the Chinese Clinical Trial Registry (registration number: ChiCTR1800016464).