Undifferentiated pleomorphic sarcoma, once called malignant fibrous histiocytoma, is a rare entity in esophageal surgery. The incidence of esophageal sarcomas, according to domestic and foreign authors, is 0.3% of all malignant neoplasms of the esophagus. In the domestic literature, we have not found any cases of radical surgical treatment of undifferentiated pleomorphic sarcoma of the esophagus. The article presents a rare clinical observation of a successful case of treatment of undifferentiated pleomorphic sarcoma of the esophagus in a 51-year-old patient. When visiting the hospital of the Regional Clinical Oncology Center in Ulyanovsk, the patient had grade 1—2 dysphagia. Upon further examination, esophageal sarcoma was verified; no evidence of distant metastasis was obtained. Surgical treatment including hybrid esophagectomy according to McKeown was performed as planned. During a routine histological examination in two centers, the conclusion was obtained: undifferentiated pleomorphic sarcoma of the esophagus. Undifferentiated pleomorphic sarcoma of the esophagus is a rare nosological form in which radical surgical treatment provides acceptable oncological results.