The clinicopathological manifestation of mucoepidermoid carcinoma of the esophagus are reported in two cases. Barium swallow and endoscopy revealed infiltrating and constricting appearances. The tumors were grossly indurated, with or without ulceration. Histologically, the tumors were composed of a mixture of glandular cells which contained signet ring cells and squamous cells. In two cases, the tumor cells invaded into the adventitia, with lymph node metastasis. Two patients died of wide-spread metastasis within 1 year after operation. Our results and previous reports suggest that mucoepidermoid carcinoma of the esophagus is extremely aggressive and has a poor prognosis.