Sixteen adult patients with congenital esophageal cysts were operated on between 1957 and 1979. Preoperatively, 7 patients (44%) were asymptomatic and the cyst was found incidentally on a routine chest roentgenogram. Esophageal symptoms were present in only 3 patients (19%), whereas most symptomatic patients had precordial sensations or arrhythmias. A correct preoperative diagnosis was made in only 1 patient. After enucleation of the cyst, preoperative symptoms were alleviated in all patients and short-term results were excellent. However, long-term follow-up 13.2 +/- 5.6 (+/- standard deviation) years later revealed moderate reflux in 9 (64%) of the surviving 14 patients. During esophagoscopy, macroscopic esophagitis was found in 12 (92%) of 13 patients. On histological examination of specimens obtained by forceps biopsy, esophagitis was present in 10 (77%) of 13 patients and included Barrett esophagus in 2. We conclude that, despite excellent early results, long-term follow-up of patients who undergo operation for congenital esophageal cysts is indicated because of the increased incidence of reflux esophagitis.