Introduction and importanceCongenital Lobar Emphysema is a rare cystic lesion of the lung which may be misdiagnosed and managed as pneumonia or pneumothorax. Case presentationWe presented a case of a congenital cystic lung malformation, the case demonstrated a 29-day-old boy who presented with respiratory distress. He was initially diagnosed as pneumonia and was given unnecessary antibiotic treatment in another hospital without improvement. Subsequently, the diagnosis of congenital lobar emphysema of the left upper lobe was made on the basis of the clinical and radiologic features. The condition wasn't detected before birth. He underwent a successful left thoracotomy with left upper lobe lobectomy. Clinical discussionCongenital Lobar Emphysema poses a challenge in diagnosis. It may mimic other causes of respiratory distress. ConclusionCongenital Lobar Emphysema requires a high index of clinical and radiological suspicion to make an early diagnosis so that timely treatment will be offered.

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