A BSTRACT Background: Urinary tract infection (UTI) is a common infection in children with nephrotic syndrome (NS). Clinical experience suggests that childhood nephrotic syndrome is frequently diagnosed incorrectly and managed inadequately on the top of this existing UTI in the episode becomes an additive obstacle for the primary care physicians or pediatricians towards optimum management, leading to poor outcome. So, we have conducted this clinico- microbiological study of UTI in NS in children to provide the exact picture of UTI with NS so that the primary care providers can be helped in having high index of suspicion of this infection and knowing prevalent organisms and their antimicrobial sensitivity pattern. Aim: The aim of the study was to study clinical features and identify the responsible organisms with its drug sensitivity pattern with response to treatment in various types and stages of NS with UTI in children. Methods: This cross-sectional hospital based study was conducted on 50 children of 2-18 years of age with NS attending nephrology clinic or admitted to the Paediatric ward of AIIMS, Rishikesh. Demographic, clinical, and microbiological data were recorded and details were entered in a predesigned proforma sheet. Results: Out of 50 cases, 8 (16%) had a positive urine culture. Six (75%) out of them had first episode and two (25%) were frequent relapsers of NS. Fever, decreased urine output, and generalized edema were the presenting features. The most common bacteria responsible for UTI was Pseudomonas aeruginosa (in around 25% isolates). Escherichia coli and Citrobacter koseri were the most resistant organisms. Patients were treated with antibiotics according to sensitivity pattern which resulted in resolution of symptoms and repeat urine culture became sterile subsequently. Conclusion: About one-sixth of children with Nephrotic Syndrome had UTI. UTI should be ruled out in every case of NS in active phase to prevent long-term morbidity and mortality.