A 78-year-old female patient with fever and general malaise was referred to our hospital. Laboratory examination showed the marked elevation of leukocyte and serum granulocyte-colony stimulating factor (GCSF) concentration without any infectious sign. A computed tomography scan demonstrated irregular enhanced mass of the right kidney with liver metastasis. The pathological findings of the needle biopsy was high-grade urothelial cancer with positive staining for G-CSF antibody. Systemic chemotherapy with gemcitabine and cisplatin was administered. The patient showed a partial response and the serum G-CSF level was normalized after 1 course of chemotherapy. After four courses of chemotherapy, the extent of liver metastasis increased and the G-CSF concentration became elevated. Although combined chemotherapy with paclitaxel and gemcitabine was administered, the patient died 7 months after her first visit.