Purpose: The diagnostic usefulness of fetal echocardiography in congenital heart disease is well known. One of the indications for fetal echocardiography is presence of extracardiac anomalies and such coexistent congenital heart disease may have important implications for obstetric and neonatal management. Materials and methods: 496 women with singleton pegnancies with amgenital anomalies that underwent penatal care and follow-up in Severance Hospital for 5 years from 1991 to 1995 were evaluated for the incidence of congenital heart disease. Results: The incidence of congenital heart disease in fetuses with the associated extracardiac anomalies were as follows; central nervous system 6.9%(11/160), gastro-intestinal system 10.7%(8/75), genito-urinary sysem 4.7%(4/85), others 5.6%(4/72). Thirty-one of eighty-ane fetuses with congenital heart disease were found to have the associated extracardidac anomalies and ten of them chenoscenal abnormalities. Conclusion: We recommend that fetuses with congenital extracardiac anomalies sbould be checked for the presence of congenital heart disease and chmmosomal abnormalities, if needed.