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Renal insufficiency is a distinct possibility in patients with congenital lower urinary tract abnormalities even though they are managed right from early childhood. Previously, such patients were not considered candidates for renal transplantation due to the fear of abnormal bladder affecting the graft kidney too. In the current era, it has been shown that the graft and patient survival rates are similar in such patients to those with normal lower urinary tract, if the urological abnormalities are managed well, before the transplantation. We present a case of successful renal transplantation in a pediatric patient with renal insufficiency due to pure urogenital sinus.