Primary malignant melanoma of the esophagus is an extremely rare tumour with a fatal prognosis. Only 122 cases have been reported to date in the literature, of which 105 are undoubtedly primary. This study presents three new cases which were surgically treated: two cases underwent subtotal esophageal resection and intrathoracic esophagogastroplasty, and one case underwent total esophageal resection and cervical esophagogastroplasty. The histogenesis, role of immunochemical markers and the therapeutic problems related to malignant esophageal melanoma are discussed in the light of a new review of the literature.