ABSTRACT
Congenital nephrotic syndrome is a severe debilitating problem associated with extra renal manifestation such as diverse cardiac findings. During four years 6 cases diagnosed as congenital nephritic syndrome in base of definitive criteria their documents reviewed and echocardiographic evaluation has been done for all with or without cardiac sign or symptoms, results gathered and expressed as incidence. All cases have some grades of structural or functional defects from simple form like as tricus-pid regurgitation to complex defects. It may be run in consecutively in siblings of a family from non consanguine parents. Pulmonary stenosis may occur in all parts subvalvualr, valvular and peripheral parts of pulmonary artery, left ventricular hypertrophy and mitral regurgitation observed in some, moderate tricuspid regurgitation observed in half of cases due to pulmonary hypertension or right ventricular hypertrophy due to pulmonary stenosis