Verrucous carcinoma of the esophagus is an extremely rare entity. It is associated with severe epithelial hyperplasia and parakeratosis of the epithelial layers. Superficial biopsies are usually falsely negative for malignant cells, as the biopsied mucosa usually demonstrates the hyperplastic epithelium. The tumor spread is through submucosal and paraesophageal infiltration. Endoscopically, verrucous carcinoma appears as a wartlike, exophytic lesion, hence the name verrucous. The diagnosis can be difficult to make, even after repeated endoscopic biopsies. We report a rare case of verrucous carcinoma of the esophagus in a 45-year-old man whose final pathologic diagnosis was confirmed only after an Ivor-Lewis esophagectomy was performed.