Background: Data on Protein Z (PZ) level may be useful in neonatal respiratory distress syndrome (RDS) as in RDS the defect in both coagulation and fibrinolytic systems may play a role in the pathogenesis and progression of the disease. Aim: To evaluate the plasma protein Z levels in neonatal respiratory distress syndrome. Methods: This study was performed on 40`neonates having respiratory distress syndrome and 40 age and sex matched apparently healthy neonates as controls. Plasma Protein Z levels were assessed in plasma of all neonates by ELISA. Results: There was statistically significant decrease in plasma protein Z level among patients than controls. In addition, there was a significant decrease in PZ among died than survived patients. A significant negative correlation was observed between PZ and severity (down score) while a significant positive correlation was observed between PZ level and gestational age. Conclusion: Plasma Protein Z. level is decreased in neonatal respiratory distress syndrome and it may be a good biomarker for detection of the severity and prognosis of the disease.