Simultaneous occurrence of clear cell variant of renal cell carcinoma and high grade papillary urothelial carcinoma of urinary bladder is a rare entity. The review of different literatures revealed incidence of carcinoma of urinary bladder and the prostate in patients with renal cell carcinoma, especially the papillary subtypes.Here we report a case of 61 years male patient presented with hematuria of one month duration. Further investigations revealed SOL in upper pole of left kidney measuring 4x4x3.5 cm and a separate friable growth in theurinary bladder. Urinary Bladder tumorspecimen was diagnosed as high grade papillary urothelial carcinoma of urinary bladder and left radical nephrectomy specimen was diagnosed as Clear cell variant of renal cell carcinoma. The etiopathogenesis of this association remains debated. It seems to be based on genetic alterations that have not been formerly proved.