A case of renal oncocytoma associated with acquired cystic disease of kidney (ACDK) in a 56-year-old man is reported. He had received hemodialysis for 15 years because of chronic renal failure. Computed tomography (CT) was performed because of distention in the upper abdomen, revealing a right renal tumor. He underwent laparoscopic right nephrectomy, and was diagnosed with renal oncocytoma. There have been reported 8 cases of renal oncocytoma in hemodialysis patients, and our case was the third one associated with ACDK in the literature.