Case Study: Autosomal dominant polycystic kidney disease (ADPKD) is an important cause of end-stage renal disease. Pretransplant native nephrectomy is performed to create space in the pelvis, to decrease compression by the enlarged polycystic kidney, and to prevent development of various symptoms. A 63-year-old man with end-stage kidney disease due to autosomal dominant polycystic kidney disease planed to deceased donor kidney transplantation. Because of massive enlarged kidneys, he underwent a bilateral nephrectomy. The diameter of the right kidney was 22*17cm and left kidney 22*16cm. Pathology indicated multifocal renal cell carcinoma in both kidney with Fuhrman nuclear grade 3/4 and no lymphovascular invasion. This case reinforces the importance of considering the possibility of an occult malignancy in the native kidneys of patients with ADPKD. we present a case of incidental renal cell carcinoma in a patient with ADPKD who underwent bilateral native nephrectomy for deceased donor renal transplantation.