A leukemoid reaction is usually associated with malignancies of the lung, stomach, and thyroid. In contrast, urothelial cell carcinoma is rarely associated with leukemoid reactions, with few cases reported over the past 30 years. Here, we describe a patient with urothelial cell carcinoma who exhibited a leukemoid reaction. The patient had an elevated white blood cell count and experienced a rapid and aggressive clinical course, terminating in death. For urothelial cell carcinoma patients exhibiting a leukemoid reaction, removal of the inciting tumor is the definitive treatment. However, considering the aggressive nature of these tumors, if the patient is unsuitable for radical surgical management, palliative chemotherapy should be considered.