Carcinosarcoma of the esophagus is a rare malignant neoplasm. It often presents as a large intraluminal polypoid tumor with early onset of symptoms resulting in prompt diagnosis. We present here the first case of a carcinosarcoma of the esophagus that had a basaloid squamous cell carcinoma component in addition to the osteosarcoma and without a transitional zone. A 57-year-old man was diagnosed with a polypoid tumor in middle third of the esophagus causing dysphagia. Local recurrence and solitary pulmonary metastasis occurred 16 and 30 months after initial excision of the tumor, respectively. Prompt and aggressive surgical resection is mandatory for carcinosarcoma of the esophagus.