Background <p>Congenital alveolar over distension, most commonly referred to as congenital lobar emphysema, is a rare lung malformation characterized by over inflation of one or more lobes of the lung. A condition that, despite its potentially life-threatening nature, is often misdiagnosed due to its radiological resemblance to more common conditions such as pneumothorax. Most commonly, the left upper lobe is involved in 40 to 50 percent of the cases. Literature indicates that timely diagnosis using CT imaging is essential, yet many cases still go unrecognized until inappropriate interventions occur. Our case demonstrates this diagnostic challenge, presenting with a rare radiological mimicry involving thin-walled cysts resembling pneumatoceles, making it a uniquely difficult and novel presentation.</p> Case presentation <p>We are reporting the case of a 3-month-old female child who presented with complaints of fever and cough for 5 days and respiratory distress for 2 days. She had a history of similar illness in her neonatal period as well. Initially, the child was misdiagnosed as having left-sided pneumothorax, and a tube thoracotomy was done, which was removed later after promptly diagnosing Lobar Emphysema on a CT scan of the of the chest. A left upper lobectomy was performed with initial uneventful recovery; however, the patient developed septic shock postoperatively and did not survive.</p> Conclusions <p>This case report helps identify gaps in the diagnostic accuracy and treatment of Congenital Lobar Emphysema with the help of early imaging, such as CT scans as recommended to differentiate it from other complex medical conditions and avoid undergoing interventions like chest tube thoracotomy procedures.</p>