A 21-year-old man presented with low back pain, left leg weakness, and walking difficulties. Magnetic resonance imaging of the lumbar spine revealed an intraspinal intradural extramedullary tumor at the level of L1–L3, causing posterior cord compression. The lesion had a signal identical to that of the subcutaneous adipose tissue on T1and T2weighted images, and suppressed on fat-saturated images (Fig. 1). It did not enhance on postcontrast images (Fig. 2). These magnetic resonance imaging findings allowed the diagnosis of spinal intradural extramedullary lipoma. Besides the tumor, there was a low-lying conus terminating at level L5–S1 and spina bifida of L5 and S1 (Figs. 1–3). Surgery was performed, and the lipoma was completely removed. Pathology confirmed the diagnosis. Intradural extramedullary lipomas of the lumbar spine are rare, and may cause symptoms related to mass effect and secondary compressive radiculopathy. These lesions may be a manifestation of spina bifida and a common cause of the tethered cord syndrome.