Crigler-Najjar Syndrome type I is a rare congenital disease with high mortality and morbidity rates due to brain complications. It has been treated by life-long phototherapy until the era of liver transplantation. Liver transplantation is currently the only curative treatment for this syndrome. Liver transplantation prevents the severe neurological complications that are the main cause of life-long disability in Crigler-Najjar Syndrome type I. The ideal age for transplantation is 3-5 years. Despite the advent of auxiliary transplantation we believe that orthotopic liver transplantation is the optimal treatment and the ideal method of liver transplantation for Crigler-Najjar Syndrome type I.