Acquired renal cystic disease has been reported in the kidneys of patients with end stage renal disease who have been untreated, treated with dialysis or undergone renal transplantation. The incidence of renal tumor development in patients with acquired renal cystic disease is greater than 5% in those undergoing chronic hemodialysis. Recently, cases of renal cell carcinoma occurring with acquired renal cystic disease of the native kidneys of transplant recipients have been reported. Transplant patients have an increased incidence of malignancy when compared to the general population. Including our case, 19 cases of de novo renal neoplasms developing in renal allografts have been reported. To the best of our knowledge we report the longest interval to presentation of a de novo renal tumor in an allograft kidney (228 months) and the first case of renal cell carcinoma associated with acquired renal cystic disease in an allograft kidney.