Severe hyperbilirubinemia in a pregnant mother suffering from Crigler-Najjar syndrome type II is a threat to the unborn child and may result in brain injury. We report the case of a Gly71 →Arg/Tyr 486 →Asp homozygous mother of East Asian descent, who was treated with phototherapy during embryogenesis and with phenobarbital during the rest of the pregnancy. This resulted in significantly reduced bilirubin levels in the mother, who gave birth to a healthy boy. A neonatal hyperbilirubinemia resolved spontaneously. Severe hyperbilirubinemia in a pregnant mother suffering from Crigler-Najjar syndrome type II is a threat to the unborn child and may result in brain injury. We report the case of a Gly71 → Arg / Tyr 486 → Asp homozygous mother of East Asian descent, who This resulted in significantly reduced bilirubin levels in the mother, who gave birth to a healthy boy. A neonatal hyperbilirubinemia resolved spontaneously.