Background: Glucose-6- phosphate dehydrogenase (G6PD) deficiency is a common inherited disorder in Iran and may cause neonatal jaundice in some conditions. This study aimed to evaluate the distribution of ABO blood groups and Rh in G6PD deficient newborns in Iran, Mashhad. Materials and Methods: This case-control multi central study was conducted on 150 icteric newborns who admitted to the NICU of educational hospitals in North-East state of Iran, Mashhad. G6PD deficiency was evaluated and case and control groups were considered of 50 icteric newborns with G6PD deficiency and 100 icteric newborns with normal levels of enzyme. Distribution of ABO blood groups and Rh were evaluated in G6PD deficient newborns and compared to controls. The prevalence of hemolysis was compared in two groups as well. Results: Prevalence of hemolysis was 22% in case group and 19% in controls. There was no significant relationship between G6PD deficiency and hemolysis (p>0.05). Distribution of blood group A was higher in case group compared to control group (68% vs. 23% respectively), the difference was significant (Z=2.55, p=0.0054). Distribution of positive Rh blood group was higher in case group compared to control group (95% vs. 85% respectively) and the differences were not significant (p>0.05). Conclusions: There was a significant relationship between distributions of blood group A in G6PD deficient newborns.