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<p>Kidney and inferior vena cava abnormalities with leg thrombosis is a newly described entity in the literature termed KILT (kidney and inferior vena cava (IVC) abnormalities with leg thrombosis) syndrome. We present a case of newly diagnosed KILT syndrome in an 11-year-old girl presenting with pain and problems with ambulation. We also review the few cases of KILT syndrome previously reported in the literature.</p>