Abstract: Objective To study the clinical features, diagnosis and prognosis of malignant melanoma in esophagus. Methods Clinical data about 9 patients with surgically-removed malignant melanoma in esophagus were retrospectively analyzed. The patients were followed up. Results The mean age of the 9 patients (8 males and 1 female) was (59.7±7.42) years. The tumor was located in the middle and lower esophagus of 2 and 7 patients, respectively. The tumor was misdiagnosed as other esophageal cancers in 5 patients by gastroscopy. Of the 9 patients, 1 underwent esophagectomy, the remaining 8 patients underwent combined esophagectomy and esophagogastrostomy. Five of them received adjuvant chemotherapy and radiotherapy after operation. The mean survival time of the patients was (7±2.34) months after operation. The average time from the definite diagnosis of tumor metastasis to the death of patients was 2.42 months. Conclusion Primary malignant melanoma of esophagus is a rare tumor with no specific clinical symptoms and imaging signs. Its clinical prognosis is poorer than that of squamous cell esophageal carcinoma and its endoscopic misdiagnosis rate is high. Surgical treatment can effectively relieve its clinical symptoms. However, no sufficient evidence can prove that surgery treatment is better than conservative medical treatment in prolonging the survival time of patients with malignant melanoma accompanying DM.