The aim of this paper is to point out the significance of the rare urinary system anomaly such as horseshoe kidney. Tumors of the horseshoe kidney are rarely described as single cases in literature. Bilateral tumors of the horseshoe kidney can present a health and therapeutically challenge. In this paper we have analyzed characteristics of the horse shoe kidney tumors and their diagnostics. Primary means of the horseshoe kidney tumors treatment are surgical with different techniques depending on the size, presentation of the tumor and other indications. The review of the available literature is also included.