Objective To investigate the surgical treatment of children with lipomyelomeningocele treated in Department of Neurosurgery, The First Affiliated Hospital of Xinjiang Medical University, from January 2013 to April 2017.Methods A total of 35 children with lipomyelomeningocele (aged one day to 10 years) who were admitted to our hospital from January 2013 to April 2017 were enrolled, and magnetic resonance was performed for all children. The Hoffman functional grade for lipomyelomeningocele was used to evaluate clinical outcome. A total of 21 children were followed up for 6 months to 3 years.Results All 32 patients achieved incision healing by first intention; of all patients, 3 experienced cerebrospinal fluid leak after surgery and underwent surgical repair immediately, and then they showed good recovery and did not experience complications such as intracranial infection. A total of 29 patients had normal neurological function before and after surgery; 5 patients with neurological symptoms showed no deterioration of neurological function after surgery; one patient with neurological symptoms showed an increase in neurological functional grade after surgery. The results of follow-up showed that among 18 patients with normal neurological function before surgery, 6 maintained normal neurological functions during follow-up, with a mean age of 7.16 months at the time of admission; 3 patients with neurological symptoms had a mean age of 42.33 months at the time of admission.Conclusions Early surgery is the key to the treatment of lipomyelomeningocele, and more attention should be paid to the abnormal changes of the skin in the lumbosacral region, in order to improve the surgical outcome.