Encephalomyelocele is a post-neurulation defect that occurs between the 8th and 12th weeks of gestation. Congenital lobar emphysema is a respiratory malformation characterized by hyperinflation of one or more lobes of the lung. External auditory canal agenesis is a rare malformation that can be a component of many syndromes. In our patient, who was diagnosed with encephalomyelocele in the antenatal period, external auditory canal agenesis was found on postoperative brain magnetic resonance imaging. Additionally, on the 34th postnatal day, the patient presented with respiratory distress, initially considered to be pneumothorax, but further investigation revealed congenital lobar emphysema. In this case presentation, we aimed to draw attention to the concomitant rare anomalies in a patient diagnosed with encephalomyelocele in the antenatal period.

Keywords: encephalomyelocele, newborn, antenatal, auditory, respiratory distress