An uncommon association of renal cell carcinoma in a pelvic kidney is described, the literature is reviewed.A case of renal cell carcinoma in a pelvic kidney is presented, and the literature briefly reviewed, with special reference to six cases reported.Renal cell carcinoma in a pelvic kidney is uncommon, the paucity of previous reports of this association is surprising. Besides renal cell carcinoma is the most common malignant renal tumour in the adult and renal ectopy is relatively common more cases of this association would be expected.Renal cell carcinoma is the most common malignant renal tumour in the adult and renal ectopy is relatively common, the actual incidence among autopsy series is 1 in 900, it has been an uncommon association, with only six cases reported. The surgical approach to ectopic kidneys merits caution because of the uncertain vascular anatomy, and vascular studies may be indicated to avoid complications, the present case is important because it represent the most common urological cancers at our institution with an unusual presentation.