We report a case of granulocyte-colony-stimulating factor (G-CSF)-producing carcinoma of collecting ducts of Bellini. A 62-year-old male was admitted to our hospital with the chief complaint of high grade fever,right flank pain and general malaise. The white blood cell count and serum G-CSF concentration were elevated to 20,100/ μ l and 140 pg/ml,respectively. Enhanced thoracoabdominal computed tomography (CT) showed a right renal malignant tumor without capsule,para-aortic lymph node metastases and lung metastases. CT-guided right renal biopsy was performed. The histological diagnosis was GCSF- producing carcinoma of collecting ducts of Bellini. The chemotherapy (gemcitabine and cisplatin) and the molecular target therapy (sunitinib) were administerd but the primary lesion and metastases was progressive and serum G-CSF concentration was elevated to 229 pg/ml. He died 3 months after diagnosis.