An L-shaped kidney is a type of crossed fused renal ectopia and consists of two moieties which were originally two kidneys. It is an uncommon condition and cases of malignant tumors in such anomalies are exceedingly rare. A case of an L-shaped kidney with renal cell carcinoma is herein reported, in which a separation of both moieties was done in situ in order to perform a tumor resection. A literature review of this topic is also reported and the findings are discussed.