A review of 107 patients with Barrett's esophagus revealed three patients who concomitantly had scleroderma. Two of the three patients had pathological evidence of high-grade dysplasia of the columnar-lined epithelium, and the third patient had nondysplastic columnar-lined epithelium in the distal esophagus. Patients with scleroderma often have an incompetent lower esophageal sphincter, poor or absent distal esophageal peristalsis, and reflux esophagitis, all of which are believed to predispose to Barrett's esophagus. The importance of Barrett's esophagus is its potential for malignant transformation. Identification of such patients permits aggressive medical treatment and endoscopic and pathological surveillance.