Laryngeal atresia is generally a fatal congenital anomaly with an incidence of 1: 50,000 births. This congenital anomaly is a condition of multifactorial inheritance, in which the fetus has a dilated trachea, enlarged echogenic lungs, an inverted or flattened diaphragm, fetal hydrops, and ascites. Diagnosis is usually made when there is failure to perform endotracheal intubation in a neonate with severe respiratory distress and absence of audible cry. Here, we present a very rare case of a newborn with laryngeal atresia who had respiratory distress and was sustained for the first few minutes of life using partial ventilation via a persistent pharyngotracheal duct. We would like to draw the attention of all physicians to this issue by reporting a rare fatal case of a newborn with a congenital presentation.