A case of polypoid carcinosarcoma of the esophagus is presented. Histologically the bulk of the tumor consisted of a sarcomatous tissue having large foci of osseous and cartilagenous differentiation and infiltrating deeply the wall, whereas a superficially, invasive squamous cell carcinoma associated with insitu carcinoma was located at the base and luminal surface of the polypoid tumor. Intermingling of the carcinomatous and sarcomatous elements was found only in areas where they appeared to be collided. Ultrastructurally the sarcomatous portion contained cells with fibroblastic features but with no typical epithelial characteristics. Immunoperoxidase staining of the paraffinembedded histologic sections for keratin proteins revealed, however, some positive spindle cells indicative of epithelial nature in the sarcomatous area, but the great majority of the sarcoma cells were devoid of keratin. These combined findings strongly suggest that the sarcomatous component in our case of true carcinosarcoma is derived from mesenchymal transformation (metaplasia) of the squamous carcinoma cells. The findings were discussed in light of the previous pertinent literature. ACTA PATHOL, JPN. 34: 669–678, 1984.