Squamous cell carcinoma of renal pelvis is relatively rare and its prognosis is very poor. A 72-year-old man was introduced to our institute because of macroscopic hematuria. He had no history of urolithiasis or urinary tract infection. Excretory urography showed a nonfunctioning right kidney. Cytologic examination of urine was positive for malignant cell from squamous cell carcinoma. Preoperative diagnosis was made as right renal pelvic tumor, but it appeared to be renal tumor on the roentgenogram. Right radical nephrectomy and transurethral ureterectomy was performed. Radiation therapy was done after operation. Pathological diagnosis was squamous cell carcinoma of renal pelvis extensively infiltrating to the renal parenchyma. The patient is alive with no recurrence or metastasis for eight months after operation. Statistical analysis was made on 136 cases of squamous cell carcinoma of renal pelvis reported in the Japanese literature including our case, and this disease is also briefly reviewed.