Several lung transplantation (LTx) patients develop end-stage renal disease (ESRD) and often need a kidney transplant. Recently, the number of multiorgan transplantation cases has increased; however, no successfully combined redo lung-kidney transplantation has been reported in Korea. We present the first case of combined second redo lung-kidney transplantation in a patient with ESRD after LTx. In November 2018, a 40-year-old man with dyspnea was admitted to our hospital. Seventeen years ago, he underwent right pneumonectomy owing to refractory extensive drug-resistant tuberculosis. Four years ago, he underwent left single-LTx due to chronic respiratory failure. He was diagnosed with chronic lung allograft dysfunction and ESRD (glomerular filtration rate, <15). He underwent a second LTx that resulted in acute graft failure. Despite the empirical management, he was not responsive to treatment. He was required to use a home ventilator, but was able to maintain good muscle strength and to walk. However, regular dialysis was required. In January 2019, he underwent combined second redo lung-kidney transplantation and was discharged. At 1-year follow-up, his pulmonary and renal functions were stable without rejection. Combined lung-kidney transplantation could be an effective treatment for selective young patients with respiratory and renal failure who have undergone LTx.