We report a case of a 69-year-old man who was diagnosed to have granulocyte colony-stimulating factor (G-CSF)-producing esophageal squamous cell carcinoma, based on a histological examination of endoscopic biopsy specimens. A high serum level of leukocytes and G-CSF was noted. Moreover, immunohistochemical examination revealed that the tumor cells were positive for antibodies against G-CSF. Palliative radiation therapy was performed because of existing distant metastasis at the time of presentation, and the patient died of tumor progression 7 months after the initial diagnosis. To the best of our knowledge, only fi ve cases with G-CSF-producing squamous cell carcinoma have been described in the English literature, including our present case. Because many cytokines induced by G-CSF-producing tumors contribute to tumor growth and aggressive infl ammation, these patients might have a poor prognosis. G-CSF-producing tumor is extremely rare; however, we should consider a differential diagnosis for such disease when a patient shows a high leukocyte count with no evidence of systemic infection or hematological disease.