Patients with hypoplastic left heart syndrome without surgery dye during the first year of life. In the literature there is only one report from our institution of a patient who survived to the second decade of life without surgery. The purpose of this work is to describe a case of a 24 years old female with hypoplastic left heart syndrome, who has survived until now in functional class I of New York Heart Association without surgery. In our patient, the survival is probably related with the presence of a ductus arteriosus, an interatrial septal defect and the secondary pulmonary arterial hypertension.