We describe the case of a 22-month-old girl who presented with a severe nephritic-nephrotic syndrome associated with arterial hypertension, remained symptomatic throughout the follow-up period and met advanced renal failure at 6 3/4 years of age. The initial renal biopsy revealed a mesangiocapillary glomerulonephritis with mesangial deposits of immunoglobulins (IgA, IgG, IgM), C3 and fibrin, extending into the capillary wall. Diffuse glomerular sclerosis was observed in the second biopsy. To our knowledge there are no reports of similar cases in the literature.