Abnormal columnar lining of the esophagus is a well-recognized premalignant condition. The management of patients with high-grade dysplasia without evidence of carcinoma remains controversial. Esophagectomy is proposed by some investigators, whereas others favor follow-up endoscopy and biopsy until microinvasive malignancy is documented. We reviewed our experience with nine patients referred with high-grade dysplasia on endoscopic biopsies without evidence of carcinoma. Eight patients had the columnar lining extending orad from the cardia, and one patient had heterotopic columnar epithelium in the cervical esophagus. All were white men ranging in age from 19 to 76 years (median: 47 years). Eight patients underwent esophagectomy with colon interposition. A sleeve resection of the cervical esophagus was done in one patient. Multifocal carcinoma was found in three patients, all of whom had severe dysplastic changes throughout the columnar lining. The patient with heterotopic columnar epithelium had microinvasive carcinoma. All four patients with carcinoma had negative nodes and are long-term survivors. No carcinoma was found in the resected specimens of the remaining five patients. High-grade dysplasia is an important marker of malignancy in patients with a columnar-lined esophagus. Esophagectomy is indicated in suitable candidates since carcinoma was found in 45% of our patients.