An eight-month-old female infant with a neurenteric cyst situated in the lumbar region is presented because of its rarity and because it can mimic other neurological malformations which occur on the same site. Microscopic examination of the surgically excised cyst revealed Water-Paccini corpuscles, lymphoid follicles, intestinal mucosa and meningeal tissue which originated from two ectoderm and endoderm-germ layers.