Objectives: To study the clinical and biochemical profile and response to Prednisolone in Nephrotic Syndrome patients presenting typically and atypically. Method: A Prospective observational study was done in 40 cases of nephrotic syndrome. Detailed history of presenting symptoms, age of onset of disease, sex of child and family history was taken. Result: Mean age was 6.28±3.82 years. Male to female ratio was 1.35:1. 19 (47.5%) patients had atypical and 21 (52.5%) patients had typical presentation. Biochemical profile was similar except for S. creatinine levels which was significantly higher in atypical type (0.001) as compared to typical type (p=0.001). A total of 36 (90%) patients had infection during proteinuria out of which UTI was most common infection (27.5%). During remission, 18 (45%) patients had infection out of which URTI was most common (25%) and no infection was seen in 55% cases. All the patients received Tab. Prednisolone 6 week daily then on every alternate day for 6 weeks. In present study 52.5% showed no relapse during follow up, 37.5% had infrequent relapse, 10% had frequent relapse. Conclusion: The present study showed male predominance with variable clinical presentation and biochemical findings during hospitalization in both typical and atypical cases of nephrotic syndrome. Also rate of infection was higher during proteinuria phase while during remission infection rate was low. Study showed no difference in outcome of steroid therapy in atypical and typical cases of nephrotic syndrome. Frequent relapse was more common in patients with atypical presentation.