A case reported here is one year-old girl, who was admitted to our neurosurgical service because of gradually increasing swelling in the left lumbar region, and maldevelopment and paresis of the left leg since birth. From the level of L-2 to S-1, there was a 8 X 7 xm subcutaneous swelling of lipomatous consistency with well-defined margins. Of particular characteristics was that the swelling didn't cross the midline and was entirely located in the left paravertebral region (Fig. 1). In the spine no gap could be felt between the spinous processes. There was no evidence of hydrocephalus, but she bad marked right sided scoliotic deformity of the lumbar region, and dislocation of hip joint and club-foot on the left side. Rectum-bladder-vaginal fistula with defect of anus and hypoplasia of kidney on the left side were also detected. Skiagram of the lumbosacral spine revealed marked right-sided scoliosis at the level of L-1 and hypoplasia of left pedicles, accompanied with marked dilation of transverse diameter of spinal canal between the level of L-1 to S-2. Defect of laminae of lumbosacral spine was not definately determined. Conray myelogram showed presence of cyst protruding laterally to the left paravertebral region probably through defect of lamina on the left side at the level of L-5 (Fig 2). At operation, it was confirmed that meningomyelocele protruded out laterally through the defect of half of lamina at the level of L-5. Massive lipoma was noted inside as well as outside the dura mater. Neural elements were replaced inside the spinal canal and the dura repaired. The postoperative period was uneventful. Diagram of defect of lamina at the level of L-5 and its relationships to the meningomyelocele sac is shown in Fig. 3. Meningocele or meningomyelocele which lays in the paravertebral lumbar region is very rare (Table 2), but the possibility of this disease should always be considered when we examine the patients with lipomatous or cystic swelling in the paravertebral lumbar region, especially when the patients present the neurological symptoms or signs.