Our objective was to describe the appearance of atresia of the left-sided atrioventricular valve on the four-chamber view of the fetal heart. Twenty-four cases of atresia of the left-sided atrioventricular connection were detected over a 2-year period. Cases were analyzed according to the appearance of the four-chamber view and the connections of the great arteries. All cases were referred because the left-sided ventricle was smaller than the right. There were five different groups of cardiac anomaly detected. Associated findings varied according to the diagnostic group. It was concluded that it is possible to identify atresia of the left-sided atrioventricular connection accurately during obstetric ultrasound examination by evaluation of the four-chamber view. However, the appearance of this view varies with the diagnosis. Each diagnostic group has different implications in terms of associated extracardiac malformations and postnatal management.