The authors present an unusual case of an intradural lipoma at the thoracolumbar level causing lateral tethering of the spine, with complicated scoliosis. A 6-year-old girl was admitted with progressive change in posture detected by her parents. Thoracolumbar scoliosis was detected by physical examination and X-ray studies. Spinal magnetic resonance imaging revealed an intradural lipoma and right lateral tethering of the spine at the upper lumbar level. After surgical release of lateral tethering and resection of the lipoma, fusion was performed by the orthopedic surgery team. Radiological and intraoperative findings of this extremely rare case are discussed.