Crigler-Najer type I syndrome is a rare almost uniformly fatal form of severe unconjugated hyperbilirubinemia that is the result of a genetic defect in bilirubin conjugation. The vast majority of the bilirubin is the insoluble form and therefore is not secreted. Bilirubin production is normal; however, the excretion rate is less than 2% of normal. The usual presentation is jaundice shortly after birth with kernicteric brain damage and death occurring within 18 months of birth.