We illustrate a case of atrioventricular valve stenosis associated with insufficiency detected in fetal echocardiography performed in a pregnant woman (22 gestational weeks) of 3rd gravida. The early prenatal diagnosis demonstrated, isolated bilateral atrioventricular value stenosis and insufficiency in the absence of any other associated complex congenital heart diseases such as tricuspid atresia, hypoplastic left heart syndrome, endocardial cushion defect. However, the mother of the affected foetus was found to be normal with no risk factors. Thus, fetal echocardiography plays a pivotal role even in low risk pregnancies for early detection of prenatal cardiac anomalies that guide the obstetricians in determining the risk stratification, therapeutic strategies and prognosis.