A 51-year-old woman with polycystic liver and kidney disease had undergone renal transplantation 21 years before presentation. She had no evidence of cerebrovascular malformations. Both her father and aunt also had polycystic kidney disease. After the renal transplantation, her liver had become progressively diseased and enlarged through cystic changes. Early satiety, malnutrition, and abdominal pain necessitated a liver transplantation. The recipient’s weight at transplantation was 59 kg. A 9.1-kg liver (white arrow) was removed and replaced with a whole graft that was one tenth the weight of the diseased liver (black arrowhead). A large cyst at the dome of the native liver had to be decompressed (white arrowhead) to allow for access to the recipient’s suprahepatic vena cava. She made an excellent recovery and had normal kidney and liver function at 4 years of follow-up.