OBJECT: Untethering of a tethered spinal cord (TSC) by transecting or removing a fatty filum terminale is a relatively simple procedure that can prevent or ameliorate neurological symptoms, and the postoperative prognosis is usually good. Progressive neurological deterioration caused by recurrent tethering has been rarely reported. The authors present their experience in cases in which a sectioned fatty filum terminale has become retethered. METHODS: The authors retrospectively analyzed the surgical results of pediatric patients with fatty filum terminale-TSC treated by transection of the filum. The patients' charts were reviewed for demographic data, clinical presentation, surgical therapy, and follow-up data. RESULTS: Of the 225 children who underwent TSC release by sectioning the fatty filum from 1992 to 2005, there were 6 patients (2.7%; 3 males, 3 females) in whom the fatty filum retethered. The mean age at the first diagnosis of TSC was 5.2 years (range 2 months-12.3 years). The mean duration from the first untethering procedure to retethering was 5.4 years. The mean age at the time of retethering was 10.6 years (range 7-17.5 years). Symptoms of retethering were urinary incontinence, low-back pain, difficulty walking, constipation, leg pain, and worsening foot deformity. Patients underwent cystometrography at the time retethering was indicated by increased bladder capacity, large post-void residual volume, decreased bladder capacity, increase in filling pressure, and poor sensation of filling. Magnetic resonance imaging revealed adherence of the rostral stump of the sectioned filum to the midline dorsal dural surface. All patients underwent the second untethering procedure. Four patients improved neurologically and experienced no retethering thereafter (mean follow-up period 5.5 years). Two patients experienced additional retethering after temporary improvement following the second untethering procedure. CONCLUSIONS: Retethering of the spinal cord is a rare condition occurring after the sectioning of a fatty filum terminale. Awareness of this rare sequela is necessary for appropriate long-term management of TSC caused by a fatty filum terminale. Cystometrography is useful for detecting the lesion and confirming the diagnosis of retethering.