Due to differences in prognosis and management, it is important to subclassify esophageal carcinoma. Esophageal lymphoepithelioma-like carcinoma (LELC) is extremely rare, with only a few cases reported to date. Review of the literature revealed case reports describing lesions with similar histology. We present a 69-year-old man with a giant pedunculated-polypoid lesion of the esophagus shrinking the lumen. Endoscopic excision of the tumor was performed and final histopathological diagnosis was confirmed to be LELC. In contrast to a previous case with a more aggressive course and a recurrent lesion, our patient died of his disease within 8 months of diagnosis. Here we discuss the endoscopic and radiologic findings of the case and a review of the literature.