We describe a patient with a long history of hiatal hernia, who at autopsy was found to have an esophagus that was totally lined by columnar epithelium. To our knowledge, this is the first documented case of an esophagus totally lined by columnar epithelium in an adult. Recent articles have emphasized the acquired nature of Barrett esophagus, also known as lower columnar-lined esophagus. Several reports have shown the ascent of columnar mucosa from the lower to middle esophagus over time. The failure of the columnar epithelium lining the esophagus to revert back to squamous type, once the irritating stimulus is removed, casts doubt on the metaplastic nature of this lesion. We believe that Barrett esophagus in a condition characterized by persistence of fetal-type epithelium and that our case represents such an occurrence.