From 1975 to 1991, 112 patients (64 men and 48 women) were found to have an epiphrenic diverticulum. Symptoms were absent or minimal in 71 patients and incapacitating in 41. All patients with minimal symptoms were managed conservatively; 35 were available for follow-up, which ranged from 1 to 25 years (median, 9 years). None of these 35 patients had clinically significant progression of symptoms. Surgical repair was done in 33 patients with incapacitating symptoms. Achalasia was present in 8 of the surgical patients (24.2%), diffuse esophageal spasm in 3 (9.1%), hypertensive lower esophageal sphincter alone in 1 (3.0%), and nonspecific motor abnormalities of the esophageal body in 7 (21.2%). Diverticulectomy and esophagomyotomy were performed in 22 patients, diverticulectomy alone in 7, esophageal resection in 3, and esophagomyotomy alone in 1. Concomitant hiatal hernia repair was done in 6 patients. Complications occurred in 11 patients; 6 had esophageal leaks. There were three operative deaths (9.1%), all occurring in patients with abnormal manometry. Follow-up was complete in 29 patients and ranged from 4 months to 15 years (median, 6.9 years). Long-term results were excellent in 14 patients (48.2%), good in 8 (27.6%), fair in 5 (17.2%), and poor in 2 (6.9%). We conclude that operation has significant risks and is not warranted in patients with minimal symptoms because progression is unlikely. Surgical treatment, however, is advisable in patients with incapacitating symptoms because most operative survivors will have long-term symptomatic palliation.