Background: Jaundice is affecting over 60-80 percent of neonates in the first week of life. Glucose-6-phosphate DEHYDROGENASE (G6PD) deficiency, which is an important cause of pathologic hyperbilirubinemia, can lead to hemolytic anemia, jaundice and kernicterus. The present study was performed to determine the prevalence of G6PD deficiency among icteric neonates in Shirvan, Iran.Methods: This descriptive, analytical study was performed by evaluating the medical records of neonates with jaundice, admitted to the neonatal ward of Imam Khomeini Hospital of Shirvan in 2012-2013. All neonates, who were evaluated in terms of G6PD, were included in this study. Data including the clinical signs and symptoms, laboratory test results and maternal history during pregnancy were recorded in the questionnaires. The patients were divided into two groups: with and without G6PD deficiency. The recorded data were compared between the two groups, using t-test and Chi-square test. P-value less than 0.05 was considered statistically significant.Results: Among 452 admitted neonates, 16 (3.5%) presented with G6PD deficiency. There was no significant difference between the two groups in terms of birth weight, weight on admission, Coombs’ test results, hematocrit level, length of hospital stay and total bilirubin level. However, there was a significant difference between the two groups regarding reticulocyte count.Conclusion: Based on the findings, establishment of an early G6PD screening program, which can prevent further complications in neonates, seems essential, particularly in countries such as Iran where G6PD deficiency is highly prevalent.