Aims of the present study were to find out the histological pattern of atypically presenting nephrotic syndrome children and their response with oral corticosteroid therapy. This prospective study was carried out in the Paediatric Nephrology unit of Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, for a period of two years, from September 2001 to September 2003. Forty children with nephrotic syndrome with atypical presentations such as gross haematuria, renal impairment, hypertension and hypocomplementaemia were enrolled for the study. For each patient proper investigations were done to screen out infection and necessary treatment was given accordingly. Renal biopsy was done in all cases after treating infection and controlling hypertension and specimen were evaluated by light and immunofluorescence microscopy. All the patients were treated with oral prenisolone 60 mg/m2/day for 4 weeks and response were observed by heat coagulation test. In present study it was observed that mean age of presentation was 8.73+/-4.36 years. Hypertension, gross haematuria, impaired renal function and hypo-complementaemia were observed in 50%, 45%, 19% and 15% cases respectively. When histopathologies were considered, 90% cases had other than minimal change disease (MCD) and Mesangial proliferative glomerulonephritis (MesPGN) was the most common histopathology (47.5%). Regarding steroid therapy only 43.2% patients were responsive to oral prednisolone therapy within 28 days. Atypically presented nephrotic syndrome usually manifested with higher age of onset, gross haematuria, hypertension, renal insufficiency and hypocomplementaemia. Mesangial proliferative glomerulonephritis was the predominant histological type. The majority (56.8%) of these atypically presented nephrotic syndromes were resistant to steroid therapy.