This report described one patient of giant polycystic liver and polycystic kidney involving iliac fossa.Preoperative computed tomography(CT)revealed a large polycystic kidney occupying partially iliac fossa space.A decompression of lower pole of original kidney was planned for placing transplanted kidney.During total liver resection plus orthotopic liver transplantation,right polycystic kidney could move up on its own and iliac fossa space was released for placing transplanted kidney smoothly.Polycystic kidney shrunk markedly post-operation.It provided references for surgical planning of combined liver-kidney transplantation for this type of disease.