kidney abnormalities comprise about 40% of all congenital anomalies. Horseshoe kidney is found in 13 % of all kidney anomalies, and affect 0,2-0,25 % of population. Horseshoe kidney and renal cell carcinoma are associated, but is an unusual entity. There is a case presentation of number and renal fusion abnormalities’ combination, localized interaortocaval, and associated with renal cell carcinona. In the case of renal fusion anomaly, in which renal tissue is presented as an unique organ with anomaly vascularization, nephro sparing interventions for keeping the organ function are selected.