The medical records of patients at Children's Hospital and Medical Center treated for lipomyelomeningocele repair were reviewed to assess the long-term outcome of surgery. In total, 108 children were cared for between 1952 and 1987, and long-term data are available for 80 of 96 children who underwent surgery. Cutaneous manifestations and associated malformations were common. Surgical repair was performed at ages 1 week to 17 years; there was no operative mortality. Thirty-five of 38 children with normal preoperative examination were without deficits on long-term follow-up review. Bowel and bladder paralysis was present in 42 children and did not recover after release of cord tethering. Eleven children had return of symptoms and were reexplored 3 to 8 years after initial surgery. Surgical repair with release of cord tethering at the time of diagnosis is advocated, regardless of patient age.