Background: In uncommon diseases, the field of health-related quality of life (HR-QoL) has received little attention. Childhood interstitial lung diseases (chILD) are a diverse group of illnesses with a high rate of morbidity and mortality. These children's HR-QoL may be impacted by their reduced lung function, the effects of their nutrition, and the stressful nature of their medical regimens. Aim of the work: to compare the HR-QoL in chILD to an aged-matched healthy population and to determine the parameters linked to a changed quality of life. Patients and methods: This is a cross section controlled study conducted on 26 children aged from 2-16 years with chILD as well as 26 age and gender-matched healthy controls , they were selected by simple random method , during the period from October,2022 to june,2023. Clinical examination, imaging studies were done for the patients group to determine the severity grade of the disease. All participants were asked to complete the PedsQLTM 4.0 Generic Core Scale (0 to 100 points). Results: The chILD group had significantly lower total mean score of PedsQL (P= 0.000) in comparison to control group. The most significant predictors and influencing variables in the chILD group are prolonged oxygen therapy (p=0.001), radiological changes, higher Fan staging (Clinical severity score) (P=0.041), recurrent hospitalization (p=0.021), intensive care admission (P=0.006), Spo2 saturation and social class (0.040). Conclusion: Since chILD is an incurable illness that progresses slowly and does not significantly improve lung function, we should concentrate more on possibly treatable factors affecting quality of life (QOL). Doing so could result in better care for individuals with chILD. Similar to how pulmonary function tests are evaluated, QOL assessment must be given proper weight when assessing chILD patients. Key words: Health-Related Quality, Childhood, Interstitial Lung Disease Patients