Renal cell carcinoma can presents wide range of signs and symptoms, and commonly associated with paraneoplastic syndrome. Paraneoplastic manifestations are present in up to 20% of patients with renal cell carcinoma. There is convincing evidence that renal cell carcinoma tumor cells elaborate proteins that serve as mediators of endocrine (ex ; ectopic production of parathyroid hormone-related protein or erythropoietin) as well as nonendocrine paraneoplastic syndromes. A paraneoplastic syndrome may be the various clinical presentation of renal cell carcinoma in a significant number of patients, therefore mimicked other general disease. We report a case of renal cell carcinoma associated with paraneoplastic syndrome mimicking pyelonephritis.