Primary Esophageal Melanoma (PEM) is an extremely rare neoplasm, with less than 270 cases described. Although clinical presentation is similar to any other esophageal neoplasm, MEP's behavior is more aggressive and fatal in most cases. We report two new cases of MEP diagnosed through endoscopy and anatomical-pathological analysis of collected biopsies. Both samples were positive for HMB-45 and S100, so the presence of primary melanoma in other location was ruled out. The form of presentation as left miosis with no eyelid ptosis or enophthalmos should be highlighted in the first case, which was described by this early manifestation. Unfortunately, the neoplasm could not be excised when diagnosed because the thoracic artery was found to be affected through echoendoscopy. In the second case, in spite of the fact that the neoplastic extension was only local, and neoplasm was subject to transhiatal esophagectomy, the patient had multiple post-surgical complications and died nineteen days after the surgical procedure. Furthermore, bibliographic review is applied to diagnosis, treatment options, and prognosis of this exceptional neoplasm.