End stage renal disease is not an absolute contraindication for liver transplantation (LT) in patients with end stage liver disease. Actuarial patient and graft survival are comparable for children and adults who undergo LT alone and liver-kidney transplantation (LKT). The most common indications for LKT are the primary hyperoxaluria type I (PH1) and the liver and renal polycystic disease. We report a 12 years old boy with congenital hepatic fibrosis with severe portal hypertension, encephalopathy and polycystic kidney disease with end stage renal disease on dialysis that underwent LKT. During the second postoperative week, he had a biopsy-proven acute liver and renal rejection, that had a good response to corticosteroids. Thirty days after surgery, the liver biopsy was without rejection. No other complications were observed (Rev Méd Chile 2003; 131: 1309-12)