Nephrotic syndrome is a rare manifestation of renal artery stenosis, presented mainly in adulthood. This is the report of a 4 months old boy admitted for hypertension and nephrotic syndrome. Left renal artery stenosis and renal hypoplasia were documented by laboratory and imaging studies. Proteinuria improved with medical treatment, with no need to surgical repair. Blood pressure remained normal during follow up, with no need to further medical treatment.