Congenital heart disease (CHD) is one of the important groups of birth defects and contributes significantly to infant mortality. Extracardiac anomalies occur in 15-45% of cases with CHD. In this retrospective study, autopsies of cases born alive and diagnosed as CHD between 1977-2002 at Hacettepe University Ihsan Dogramaci Children's Hospital, Pediatric Pathology Department, were investigated. In this period, a total of 3320 autopsies were performed and the incidence of CHD was 9.1%. The most commonly encountered CHD was ventricular septal defect (VSD) (15.3%).