Abstract: Objective: To analyze the clinical characteristics of neonatal G6PD deficiency complicated with hyperbilirubinemia,and study the predisposing factor,clinical process,diagnosis,and treatment outcome of G6PD deficiency disease during neonatal period.Method: 12 neonates of G6PD deficiency complicated with hyperbilirubinemia were reviewed from Oct.2007 to Jun.2012,about the etiology,clinical features and outcomes.Result: The peak time of hyperbilirubinemia ranged from 2 to 7 days after birth.The maximum serum total bilirubin (STB) was (417.38±154.73) μmol/L,and eight of patients with STB>342 μmol/L(66.7%).Blood hemoglobin level (Hb) range was 52~164 g/L,mean Hb level was (123.37±34.54) g/L and Hb<140 g/L in 66.7% of patients.Blood reticulocyte (Ret) counted 1.31~4.22% and (2.50±0.94)% as average value.There was no significant correlation between STB and either G6PD/6PGD ratio,Hb or Ret (r=-0.175,-0.180,0.272;P=0.587,0.576,0.393,respectively).The comorbidities:4 cases of infectious diseases,and 1 case of asphyxia,2 cases of ABO hemolytic disease,2 cases of kernicterus.Conclusion: This study showed that neonatal jaundice induced by G6PD deficiency was characterized by early onset,rapid progression and severe hyperbilirubinemia.Most babies had no obvious trigger factors.G6PD activity and Hb level can not fully reflect the severity of jaundice or the degree of hemolysis.For neonates with early severe jaundice,we suggest that G6PD activity should be examined to exclude G6PD deficiency.