An estimated 150,000 children are born with birth defects each year. One of the most frequent genitourinary abnormalities is horseshoe kidney (HSK). The incidence of HSK in the population is estimated to be 1/400 to 1,600 births based on autopsy data from the 1940s and 1950s. We prospectively evaluated the incidence of HSK based on radiographic studies to determine the contemporary incidence of HSK.