Children with end-stage renal disease and inferior vena cava (IVC) thrombosis are rare, and their condition is complex and high risk for renal transplantation. Detailed imaging studies of the recipient's abdominal vasculature should be carried out prior to transplantation, followed by careful pre-operative joint planning by the pediatric transplant surgeon and nephrologist. Critical decisions need to be made as to whether a deceased child's kidney or an adult-sized kidney is to be used, and if the latter, whether it should be from a deceased or living donor. In addition, the contemplated site of the donor's renal vein anastomosis needs to be determined with a consideration of the possible consequences of the various choices. Sixteen cases of renal transplantation in children with pre-existing IVC thrombosis are reviewed, including the three reported by Shenoy et al. in this journal. With a full understanding of the difficulties noted, renal transplantation in a small child with IVC thrombosis can be successful. However, it requires thorough recipient assessment, coupled with a careful and thoughtful examination of options, to determine the best possible approach to the transplantation.