Acquired cystic disease of the kidney and renal cell carcinoma are associated with chronic renal failure. In recent years, there has been increased incidental detection of renal tumours through the liberal use of ultrasonography (US) and computed tomography (CT). A 40-year-old man suffering from chronic renal failure and who was being treated with haemodialysis for six years, was found to have a complex cystic lesion on US and CT. Nephrectomy was performed and the lesion was confirmed to be renal cell carcinoma associated with acquired cystic kidney disease. The current role of imaging in the management of affected patients are discussed.