In pediatric patients with end-stage renal disease, renal transplantation is the established therapy of choice. The commonest cause is a congenital abnormality of the kidneys and urinary tract, often associated with lower urinary tract dysfunction (LUTD). Historically, such patients were denied transplantation, but it is now widely accepted that transplant outcomes comparable with the non-LUTD population are achievable. Nonetheless, the optimal management of pediatric end-stage renal disease patients with LUTD is unclear, with no guidelines to distinguish between the need for conservative management or surgical reconstruction of the lower urinary tract. Furthermore, the most appropriate surgical procedure and optimal timing of surgical intervention is far from clear. In this review, we outline common conditions that produce LUTD in children; discuss difficulties encountered in assessing the need for surgical treatment; provide an overview of the surgical procedures available; and consider the evidence for and against surgical intervention before, during, and after renal transplantation.