A 14-year-old female patient presented with symptoms of chronic mid and low back pain that radiated to both lower limbs for 5 months, with rapidly progressive lower limb weakness and urine retention. Radiologic evaluation revealed an intramedullary mass at the level of first to sixth thoracic vertebrae. The patient underwent surgery and intraoperative findings showed an intramedullary mass lesion composed of well-differentiated fat tissue. A postoperative histopathologic examination revealed mature adipose tissues consistent with lipoma. Post-operatively patient showed improvement in lower limb motor power and started an extensive rehabilitation program.