Laryngotracheoesophageal cleft is a rare congenital anomaly that should be considered in the differential diagnosis of any neonatal respiratory distress aggravated by feeding. Diagnosis is difficult, but can be made by roentgenographic and endoscopic techniques. Roentgenographic evidence of abnormal positioning of nasogastric tubes should arouse suspicion. Repeated bronchoscopy may be necessary to identify the defect. Immediate surgical repair is indicated. Incorporation of part of the esophageal wall into the repair may enhance success. Tracheostomy has been required in all survivors. The embryologic pathogenesis of the anomaly is discussed and it is concluded that the defect is found more often than is reported. We present the fifty-fourth case, the first associated to hypospadias in the world, and the first case in the Mexican literature. This child presented severe respiratory distress. Death occurred on the tenth neonatal day.