Renal cell carcinoma (RCC) in autosomal dominant polycystic kidney disease (ADPKD) is very rare. Only 11 cases of bilateral RCC in ADPKD have been reported since 1954. Herein, we present a 58-year-old male who received laparoscopic bilateral radical nephrectomy for bilateral RCC with different cell variants in ADPKD and end-stage renal disease under regular hemodialysis.