G-CSF producing esophageal carcinosarcoma is extremely rare, and its effective treatment strategy remains undefined. Here, we report the case of a 69-year-old woman who underwent successful two-stage surgery using mediastinoscopic esophagectomy and laparoscopic reconstruction for the management of severe anemia, malnutrition, and inflammation due to G-CSF producing esophageal carcinosarcoma(G-CSF 265 pg/mL). Chemoradiotherapy could not manage lymph node recurrences in the patient; however, nivolumab was found to be effective and helped achieved a prolonged partial response.