Congenital lobar emphysema (CLE) is an uncommon congenital malformation of the lung that can present with respiratory distress in early life. We report a full term female baby who presented with intermittent bluish discoloration of the lips and extremities during crying since birth. An initial diagnosis of left sided collapse-consolidation with right-sided compensatory emphysema was made from chest x-ray. When no improvement was noted after intravenous antibiotic therapy for 7 days, computerized tomographic scan of thorax was done which showed emphysema of the right middle lobe with transherniation and mediastinal shift to the left. Right middle lobectomy was done and postoperative period was uneventful. Histopathological examination of the specimen confirmed the diagnosis of CLE. CLE can mimic more common causes of respiratory distress in neonatal period and needs a high index of suspicion for diagnosis. Early diagnosis and effective surgical treatment is curative.
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