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Unilateral pulmonary hypoplasia is a congenital condition characterized by underdevelopment of one lung during fetal growth. The causes of this rare condition may involve genetic factors, vascular issues, or other structural anomalies. Symptoms and complications can vary in severity, ranging from respiratory distress in newborns to recurrent respiratory infections and growth challenges in children. Diagnosis relies on prenatal ultrasounds and postnatal imaging, with treatment options encompassing supportive care, surgery, and respiratory therapy. Timely intervention is essential for improving the respiratory function and overall well-being of affected individuals. A better understanding of the genetic and developmental aspects of this condition may pave the way for enhanced treatments in the future.