BackgroundEnd-stage renal disease is a risk factor for renal cell carcinoma. However, mucinous tubular and spindle cell carcinoma of the kidney is rare. We report a patient on hemodialysis who had high-grade mucinous tubular and spindle cell carcinoma of the kidney.Case presentationA 62-year-old Japanese woman had end-stage renal disease and had been on hemodialysis for the previous 4 years. However, periodic imaging examinations to detect kidney cancer had not been conducted. The patient visited our department of urology complaining of general fatigue and dyspnea. Computed tomography with enhancement revealed a solid renal mass with a diameter of 66 mm. It had invaded the right psoas muscle and had multiple lymph node metastases. In addition, the patient had massive ascites and right-sided predominant pleural effusion. We drained the pleural effusion, but she died 11 days after admission. An autopsy was performed. Pathological examination revealed high-grade mucinous tubular and spindle cell carcinoma of the right kidney; multiple organ metastases to the bilateral lungs, liver, gall bladder, uterus, ovary, and inferior vena cava; cancerous pleuritis; and cancerous peritonitis. Our case indicates that high-grade mucinous tubular and spindle cell carcinoma emerging in end-stage kidney causes a detrimental clinical course.ConclusionHigh-grade mucinous tubular and spindle cell carcinoma of the kidney had a severe clinical course. We recommend periodic screening of patients with end-stage renal disease, using computed tomography or ultrasound.