Renal cell carcinoma is the most feared complication in patients with acquired renal cystic disease. We present a 73-year-old man with end stage renal disease in chronic hemodialysis for eleven years that was admitted to the hospital because of hematuria and right flank pain. Two months before admission the abdominal CT reported atrophic changes of the right kidney with multiple masses. A right laparoscopic nephrectomy demonstrated renal cystic disease associated with multifocal cystic renal cell carcinoma, clear cell, nuclear grade II. There was no extra renal extension or renal pelvis involvement. Patients with end-stage renal disease who have been on hemodialysis for three to five years should undergo yearly screening with ultrasonography. Once the ultrasonogram becomes positive for cysts, the more sensitive contrast enhanced CT scanning should be performed yearly to screen for the possible occurrence of renal cell carcinoma.