A case of a giant esophageal pedunculated lipoma diagnosed in a 67-year-old male patient with recent onset of dysphagia is reported. Following the initial endoscopic and radiologic evaluation, the diagnosis was suspected by endo-ultrasonography and confirmed by histopathology. The treatment consisted of an esophagotomy with submucosal resection, through a left sided cervical incision. The review of the literature confirmed the rarity of this neoplasm, and the difficulty of a correct preoperative diagnosis, since a possible progression to squamous carcinoma has been demonstrated.