The purpose of this report is to describe a new case of primary malignant melanoma of the esophagus (PMME) and to review the recent literature. A 75-year-old man underwent an esophagoscopy for a 3-month history of dysphagia and weight loss. A pigmented polypoïd mass in the lower third of esophagus was discovered, identified by biopsy as a malignant melanoma. No pigmented lesions of the skin or eyes were observed and a diagnosis of PMME was made. A total transhiatal esophagectomy was carried out and 12 months after the operation the patient is disease-free. PMME is a rare neoplasm, with only 238 cases having been reported in the literature. Although characterized by an aggressive biological behavior, esophagectomy can result in a 5-year survival rate of up to 37% of cases, whereas chemotherapy, immunotherapy and radiation therapy currently have no major role in treatment.