Congenital heart disease (CHD) is the leading cause of infant mortality due to congenital defects in the world. A retrospective cross-sectional descriptive study was conducted with the aim of characterizing congenital heart disease in children under one year of age at the Policlínico Universitario XX Aniversario in the period January 2015 December 2018. The population and sample consisted of 75 children born with congenital heart disease. For the collection of information, a documentary review was performed and statistical methods of absolute and relative frequencies were used. Pathological antecedents of congenital heart disease in first line relatives predominated; anemia, threatened abortion and cervical-vaginal infections were frequent during pregnancy, as well as the low use of folic acid. A high percentage of the mothers consumed coffee, followed by smoking. Congenital heart disease in children under one year of age predominated in the female sex. Atrial septal defect, followed by patent ductus arteriosus and ventricular septal defect were the most frequent congenital heart defects.