Experience with horseshoe kidney transplantation is limited. Horseshoe kidney may be underutilized for transplantation because of the greater incidence of vascular and other associated urological anomalies. Nowadays, owing to a greater number of patients waiting for a kidney donation and to a shortage of organs donated, more suitable organ acceptance criteria have been formulated. The aim of this paper is to present the first Iranian experience with horseshoe kidney transplantation.