Left ventricular hypoplasia is characterized by a variable egree of undevelopment of the left ventricle and its components, being considered a surgical emergency. This situation can progress to respiratory problems, increasing acisosis and circulatory collapse. Esophageal atresia (LA) with or without tracheoesophageal fistula is the most commun congenital anomaly of the esophagus and is characterized by abundant salivation, episodes of cyanosis and asphyxia during breastfeeding. This article amins to report a case of a newborn with hypoplastic left ventricular syndrome associated with esophageal atresia