Congenital lobar emphysema (CLE) and congenital pulmonary lymphangiectasis (CPL) are rare conditions that are most often identified with prenatal ultrasonography. Occasionally, this disease process is first identified in the emergency department (ED), where the physician should avoid common pitfalls in order to prevent acute decompensation. To the best of our knowledge, there are no prior reports in the emergency medicine literature of CLE or CPL presenting to the ED as undifferentiated respiratory distress in an infant. Here, we describe one such case and then discuss the importance of differentiating these congenital anomalies from more commonly encountered emergency diagnoses, such as pneumothorax and pneumonia. Management differs radically, and the use of chest tubes and positive pressure ventilation in CLE may precipitate acute cardiovascular decompensation.