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.