Immunoglobulin A (IgA) nephropathy is considered to be the commonest primary glomerulonephritis worldwide. The commonest clinical presentation of the disease is macroscopic hematuria, and nephrotic syndrome (NS) at the time of onset of symptoms is a predictor of poor outcome in both adults and children. In this report we describe a case of IgA nephropathy in a 15-year-old girl with a diagnosis of NS who was admitted to our hospital following 15 days of remarkable weight gain, and eyelid and pretibial oedema. NS was diagnosed when she was 3 years old. During the period between diagnosis and our observation the patient presented with three episodes of relapse. After the third episode a renal biopsy was performed and, together with clinical data, this enabled us to make the diagnosis of IgA nephropathy. NS is now recognized as a possible clinical manifestation of an IgA nephropathy, even at onset. Based on this case report, we suggest that a more vigilant management of children with NS may be necessary, even if they do not have atypical characteristics at onset. We suggest that it may be better to perform a renal biopsy at the time of first relapse.