Abstract: Objective　To analyze the Uridine diphosphoglucuronyl transferase 1 A1 gene (UGT1A1) mutation in non-hemolytic indirect hyperbilirubinemia. Methods　A female patient was diagnosed with type I Crigler-Najjar syndrome (CNS-I) after excluding hemolysis and hypothyroidism. Phototherapy treatment is effective while oral phenobarbital was not. UGT1A1 were amplified by polymerase chain reaction and DNA was sequenced. Results　The patient was compound heterozygote of c.1070A>G p. (Gln357Arg) and 1091C>T p. (pro364Leu) and diagnosed with CNS-I. Heterozygotes of the mutation were found in her parents. Conclusions　In patients highly suspected of CNS, the genetic tests should be carried out as soon as possible.