A rare case of squamous cell carcinoma of the right kidney associated with giant hydronephrosis and right ureteral stone was reported. A 60-year-old woman was referred to our department with a complaint of ileocecal dull pain. A plain computerized tomogram (CT) revealed giant hydronephrosis, and a kidney-ureter-bladder X-ray revealed a right lower ureteral stone. A right percutaneous nephrostomy was done and antegrade pyelography revealed giant hydronephrosis and a ureteral stone, but showed no intrapelvic filling defect. Under the diagnosis of right ureteral stone with hydronephrosis, right ureterolithotomy was done. However, since the inflammation did not subside after ureterolithotomy, right nephrectomy was done. The surgically removed right kidney did not show any evident tumor mass, a histological study revealed squamous cell carcinoma involving renal parenchyma. The patient received no adjuvant therapy and died 3 months after nephrectomy with sudden appearance of bone, liver and pulmonary metastases. The preoperative efficacy of imaging modalities for renal squamous cell carcinoma was discussed herein.