A 64-year-old man with well-differentiated squamous cell carcinoma of the maxilla who presented with high levels of granulocyte-colony stimulating factor (G-CSF) is described. The extent of the tumor was staged as T4N1M0. Regional chemotherapy, radiotherapy and resection of the tumor were performed. The patient died of local recurrence and lung metastasis about four months after admission. During his clinical course, a peripheral blood analysis demonstrated marked granulocytosis and high G-CSF activity. G-CSF producing cancer cells were immunohistologically observed in the primary lesion and metastatic lymph nodes. Therefore, he was diagnosed with G-CSF producing maxillary cancer. The cancer tissue from this patient was transplanted into nude mice, and one of these mice demonstrated distant metastasis to the lung.