Abstract
Congenital unilateral pulmonary hypoplasia of a pulmonary artery is considered a rare congenital anomaly in the pediatric and adult population. With an estimated prevalence of one in 200,000, it can range from partial to near-total lung underdevelopment. The diagnosis of lung and pulmonary artery hypoplasia is challenging in adults as they can easily be mistaken for more common diseases. Many survive into adulthood with minimal or no symptoms, which makes their identification challenging. We present the case of a 14-year-old female with a previous diagnosis of 3-methylglutaconic aciduria (3-MGA-uria) with a history of chronic wet cough andrecurrent respiratory tract infections (RTIs) that led to multiple hospitalizations throughout her childhood. After further evaluation, the patient was diagnosed with hypoplasia of the right-sided pulmonary artery system and its branches. This case report highlights the importance of early identification of congenital unilateral pulmonary hypoplasia of a pulmonary artery to prevent pulmonary complications like recurrent RTIs in pediatric patients with rare diseases.