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Nephrotic syndrome (NS) represents a common disease in children characterized by a relapsing and remitting course associated with edema that can significantly affect their quality of life (QOL). Chronic Kidney Disease (CKD) has a major impact on a child's development. However, there is little information on the QOL of children with Nephrotic Syndrome and Chronic Kidney Disease from patient's and parents' perspective. Hence we examined the comparative QOL of children having steroid sensitive nephrotic syndrome (SSNS), steroid resistant nephrotic syndrome (SRNS), CKD and healthy siblings. Materials and methods: A comparative, cross-sectional study on 109 children using consecutive sampling was done. QOL of children was assessed by two methods: child self report and parent proxy report using Peds QL 4.0. Data was analyzed using STATA 11.2. Results: There was no significant difference in total QOL scores of children in all the four groups. However, on domain specific analysis, we found that absenteeism from school due to sickness or hospital visits was a common problem as per children with NS and CKD and their parents but not in siblings (p < 0.05). Children with SRNS reported more difficulty in running than siblings (p=0.01). Parents reported significantly less self care ability in CKD group as compared to SSNS group (p=0.04). Conclusion: The total QOL scores of children having SSNS, SRNS, CKD and siblings were similar as reported by child's self report and parent's proxy report. However, a few differences were found in some items of individual domains. [ABSTRACT FROM AUTHOR]