Carcinosarcoma of the esophagus and the stomach are rare neoplasms characterized by the simultaneous presence of carcinomatous and sarcomatous elements. There is no report in the literature of carcinosarcoma of the esophagogastric junction. We present a case of carcinosarcoma of the esophagogastric junction whose unique clinical presentation, surgical issues, morphological and immunohistochemical features makes it quite distinctive from similar cases observed in the esophagus or in the stomach.