Primary malignant melanoma of the esophagus (PMME) is an uncommon but aggressive tumor with very poor prognosis. There is no established treatment plan for the disease, which may be attributed to its rarity and aggressiveness. Surgery is the choice of treatment in early cases. Radiotherapy follows surgery, and chemotherapy has an insignificant role in its treatment. Radiation with heavy ion beams is showing promising results in cancer therapy. Compared to conventional radiation, it permits selective irradiation with minimal injury to the surrounding normal tissue, and treatment with a low dose within a short interval of time is possible. We herein report a case of PMME treated with heavy ion radiation, the first case to be reported so far, and review the relevant literature.