A case of 2-month-old girl with lumbosacral lipomeningocele was reported. She was admitted for evaluation of a large soft mass in the lumbosacral area, initially noted at birth. On examination, the tumor was 7 cm in diameter and 3 cm in height. The upper part of a mass was cystic. Plain X-ray films revealed bifid lower lumbar and sacal vertebra. Spinal CT scans revealed a mass and bifid spine. An area with EMI units, 2.4+/-4.2, at L4 level and another with EMI units, -52.9+/-5.5, at L5 level were seen extending from within the canal to the subcutaneous tissue. The former was identified as water and the latter as fat. The preoperative diagnosis of lipomeningocele was made. Operation was performed under the operating microscope. A meningocele sac contained no neural element and it's surface was adhesive to lipoma. Lipoma involved cauda equina and conus medullaris in the canal. We removed the extraspinal lipoma with meningeal sac and a part of the intraspinal lipoma to avoid neurological deficit. We consider, like many authors, that intraspinal lipoma should be operated as early as possible with the help of surgical microscope. Early diagnosis is necessary. Myelography is valuable but caution is needed at time of lumbar or cisternal puncture. But spinal computed tomography is noninvasive and allows for precise diagnosis without contrast myelography. CT scan is extremely useful in diagnosing congenital abnormality of the spine.