OBJECTIVE Lipoma of the conus medullaris (LCM) causes neurological symptoms known as tethered cord syndrome (TCS). The symptoms can be seen at diagnosis and during long-term follow-up. In this report, pediatric patients with LCMs who underwent untethering surgery, under the policy of performing surgery if diagnosed regardless of symptoms, were retrospectively reviewed to evaluate long-term surgical outcomes. Possible risk factors for retethered cord syndrome (ReTCS) were evaluated in the long-term follow-up period. METHODS A total of 51 consecutive pediatric patients with LCMs who underwent a first untethering surgery and were followed for > 100 months were retrospectively analyzed. The surgery was performed with the partial removal technique. Pre - and postoperative clinical and radiological data were reviewed to analyze the outcomes of surgery and identify potential risk factors for ReTCS. RESULTS During follow-up, 12 patients experienced neurological deterioration due to ReTCS. The overall 10-year and 15-year progression-free survival rates were 82.3% and 75.1%, respectively. On univariate analysis, a lipoma type of lipomyelomeningocele (OR 11, 95% CI 2.50-48.4; p = 0.0014), patient age at the time of surgery (OR 0.41, 95% CI 0.14-1.18; p = 0.0070), and the mean patient growth rate after surgery (OR 2.00, 95% CI 1.12-3.41; p = 0.0040) were significant factors associated with ReTCS. Cox proportional hazard models showed that a lipoma type of lipomyelomeningocele (HR 5.16, 95% CI 1.54-20.1; p = 0.010) and the mean growth rate after surgery (HR 1.88, 95% CI 1.00-3.50; p = 0.040) were significantly associated with the occurrence of ReTCS. CONCLUSIONS More complex lesions and a high patient growth rate after surgery seemed to indicate increased risk of ReTCS. Larger prospective studies and registries are needed to define the risks of ReTCS more adequately.