The aim of this study was to define the clinicopathological features and prognosis of esophageal cancer in patients 40 years and younger. Between 1990 and 2007, 549 patients with esophageal cancer were enrolled in a retrospective database. Patients were divided into two age groups: Group A was under 40 years old, and group B over 40 years old. The findings for 50 patients (9.1%) aged 40 years or less were compared with those of 499 patients over 40 years old. In the younger group, there were significantly more women (35/15 vs. 231/268, P < 0.001). In the young and old groups, the most frequent histological morphology was squamous cell carcinoma (88% and 75%, respectively), although the percentages were significantly different (P < 0.005). In the younger group, lesions were more frequently located in the middle one-third of the esophagus than in the older group (64% vs. 28.3%, P < 0.001). Group A was more likely to have Stage IIa than older patients. In both groups, the surgical removal of the tumor was the most frequently used choice (70% vs. 56.5%). Survival rates in younger patients at 5 years after resection were 11.4%, similar to those in older patients (16.9%, difference not significant). In the group under age 40, women predominated. Squamous cell carcinoma was the most common histology, followed by adenocarcinoma. Younger patients did not have improved 5-year survival compared with their older counterparts.