The authors have examined the statistics of extracardiac malformations in infants with congenital heart disease, aged between birth and 12 months. Cases have been registered and studied within a period ranging from 1969 to 1979 at A. Meyer Hospital of Florence (Pediatric Institute of the University, Divisions of Medical Pediatrics and of Pediatric Surgery). Extracardiac abnormalities occur in 23,05% of infants seen during the first year of life for significant cardiac disease. Sometimes the extracardiac malformations are multiple and 66,3% of the affected infants have some established syndrome, especially chromosomal. The most frequent associated cardiac malformations are: ventricular septal defect (29,3%), complex heart disease (18,5%), non cyanotic heart disease with increased pulmonary blood flow (16,3%) and endocardial cushion defect (10,8%).