BACKGROUND: Nephrotic syndrome (NS) is relapsing-remitting illness affecting children and characterized by proteinuria, edema, and hypoalbuminemia. The disease involves the significant formative years of a child's life and profoundly impacts their physical and psychosocial well-being. There is a scarcity of literature exploring the quality of life (QoL) of children with NS and the factors influencing them. The study aimed to discuss the QoL of children with NS and the factors influencing it. MATERIALS AND METHODS: A systematic search of electronic databases, including PubMed, Google Scholar, and Embase, was carried out from 2004 to August 2023. Nineteen original articles in English language that focused on evaluating the QoL of children with NS were included. Articles in languages other than English, letters, and editorial reviews, studies involving populations other than children, were excluded. RESULTS: Children with NS tend to have a better QoL as compared to those with other chronic diseases (p = <
0.001), but it remains lower than that of healthy children (p<
0.05). School functioning was the most affected domain. Recurrent relapses, prolonged illness, high steroid dosages, and multiple medications are significant contributors to impaired QoL. Children with steroid-resistant nephrotic syndrome (SRNS) had poorer QoL among the clinical subtypes. CONCLUSION: Children with NS face various challenges that impact their QoL. Comprehensive care strategies to enhance QoL are the need of the hour.