Purpose: To assess the effect of inhaled nitric oxide (iNO) on oxygenation in the management of pulmonary hypertension (PH) secondary to arteriovenous malformations (AVMs) in neonates.Method: A matched retrospective cohort study from January 1, 2013, to December 31, 2017. European inhaled nitric oxide registry from 43 neonatal and pediatric ICUs in 13 countries across Europe was used to extract data. The target population was neonates treated with iNO for the management of PH. The cases (PH secondary to AVMs treated with iNO) were matched (1:4 ratio) to controls (PH without AVMs treated with iNO). The main outcome measure was the absolute change of oxygenation index (OI) from baseline to 60 minutes after starting iNO in cases and control.Results: A total of 45 infants out of 1830 (9 cases and 36 controls) were identified, who received inhaled nitric oxide during ICU stay. The mean baseline value of OI (%) (cases: 27.5, controls: 34), the mean value of OI (%) after 60 minutes of iNO use (cases: 16.8, controls: 28.5), and the mean absolute change in OI value (%) from baseline to 60 minutes of iNO (cases: 10.7 and controls: 6) was not statistically different between groups.Conclusion: Infants with PH secondary to AVMs treated with iNO did not respond differently compared to babies presented with PH without AVMs treated with iNO. Right ventricle dysfunction on echocardiography was higher in cases (cases: 66.7% and controls: 28.6%) but not statistically significant. The death rate before discharge was significantly higher among cases (cases: 55.6% and controls: 8.3%).