Objective: To investigate the effect of combined liver and kidney transplantation on primary hyperoxaluria type 1 (PH1) in children. Methods: The preoperative and postoperative clinical data of a patient with PH1 who underwent combined liver and kidney transplantation in the Kidney Department of the Children""s Hospital of Nanjing Medical University were analyzed retrospectively, and the related literature was reviewed. Results: The patient was a 16 years old boy. At the age of 1, he found multiple stones in both kidneys. He had been treated with lithotripsy and stone removal in other hospitals, and the effect was poor. At the age of 10, the gene detection showed that the AGXT gene had a complex heterozygous mutation. At this time, renal insufficiency had occurred, and it rapidly progressed to end-stage renal disease within half a year. Two years after regular high flux hemodialysis in our hospital, he performed combined liver and kidney transplantation in another hospital.One week after the operation, oliguria and urinary incontinence occurred, and the serum creatinine was rechecked at 200μmol/L. After oral hydrochlorothiazide, potassium citrate and other treatments, serum creatinine maintained at 140~150μmol/L. One and a half years after combined liver and kidney transplantation, renal biopsy of the transplanted kidney was performed. Pathology showed that the transplanted kidney had critical changes. 3.5 years after combined liver and kidney transplantation, serum creatinine was stable at 140μmol/L. A total of 10 patients with PH1 received combined liver and kidney transplantation and bilateral autologous kidneys were reviewed. The patients were followed up for 1-9 years, and the renal function of the transplanted kidneys was normal. Conclusion: Combined liver and kidney transplantation is an effective method for the treatment of end-stage renal disease caused by PH1 in children. However, since the autologous kidney is the main repository of oxalate in the whole body, oxalate may be mobilized into the plasma and urine after surgery, resulting in renal dysfunction of the transplanted kidney. It may be more effective to protect the renal function of the transplanted kidney by simultaneous resection of bilateral autologous kidneys after combined liver and kidney transplantation.