Esophageal sarcomatoid carcinoma (ESC) is a rare malignancy, accounting for about 2% of esophageal malignancies. It is also referred to by numerous other names, including carcinosarcoma, pseudosarcoma, pseudosarcomatous squamous cell carcinoma, spindle cell carcinoma and polypoid carcinoma, suggesting that its pathological mechanism remains undefined. Its clinical and radiological manifestations are similar to other esophageal tumors. Barium esophagogram usually reveals the presence of a large polypoid mass in the lumen. While ESC is typically large, it has a relatively good prognosis, which may be explained by its exophytic growth rather than the infiltration into the esophageal wall. Surgical resection of the esophagus and stomach is the basis of early detection, effective treatment and long-term survival, and is the main treatment for ESC. However, as a rare disease, ESC is often misdiagnosed in clinical setting, especially in cases where a biopsy cannot provide sufficient specimens. In this article, we report a case of suspected ESC, in whom surgical treatment was avoided after international multidisciplinary consultations.