Sarcomatoid carcinoma of the esophagus is an uncommon malignancy, representing approximately 2% of esophageal carcinomas. It has also been referred to as carcinosarcoma, pseudosarcoma, pseudosarcomatous squamous cell carcinoma, spindle cell carcinoma, and polypoid carcinoma, reflecting the uncertainty of its pathogenesis. Histologically, carcinomatous and sarcomatous components coexist. The clinical and radiologic findings resemble other esophageal neoplasms. Sarcomatoid carcinoma often presents as a large, intraluminal, polypoid mass on barium esophagram. Despite its size, however, sarcomatoid carcinoma has a more favorable prognosis than other malignant esophageal neoplasms, likely because of its exophytic growth into the lumen, rather than deep invasion. This article provides a brief overview of the clinicopathologic features and possible pathogenesis of this uncommon tumor.