Primary malignant melanoma of the esophagus is a rare disease. A case is reported and the newer diagnostic techniques of immunohistologic identification of the tumor by positive reaction to the HMB-45 antigen, as well as immunoscintigraphy with Technetium-99m-labeled melanoma monoclonal antigen for the demonstration of distant metastasis, is presented. A current review of the literature on this uncommon tumor is presented, and treatment options are discussed. A total esophagectomy remains the treatment of choice. Four long-term (> 5 years) survivors after adequate surgical removal have been recorded in the literature.