The overall survival rate in infants affected by congenital heart disease remains low. The poor outcome may be related to the increased association with extracardiac anomalies and aneupoloidy. Since most types of CHD are now amenable to surgery, it is essential that any fetus with congenital structural heart defect should be completely evaluated, noting in particular congenital extracardiac defects which may adversely affect the prognosis. Noncardiac malformations have been reported to occur in up to 50% of patients with congenital heart disease. The combination of cardiac anomalies with other organ abnormalities appears in genetic syndromes and after exposure to environmental factors. Fetuses with prenatally diagnosed cardiac malformations need to have offer structural abnormalities ruled out. They should be evaluated thoroughly by ultrasound exam, because the detection of an extracardiac anomaly may dramatically affect prognosis. Also, fetuses in which an extracardiac anomaly or chromosome abnormality is diagnosed deserve a complete evolution with full fetal echocardiography. Fetal caryotype analysis should be performed since the risk of aneuploidy approaches 40% in cases of prenatally diagnosed CHD.