The paper presents a case study of an infant with symptoms of gastroesophageal reflux, recurrent pneumonia, and tracheal narrowing observed during laryngoscopic examination, in whom intralobar sequestration was suspected during the diagnostic process. The article further provides an overview of current knowledge concerning the aetiology, clinical presentation, therapeutic approaches, and complications of pulmonary sequestration. Pulmonary sequestration is a rare congenital anomaly of the respiratory system in which non-functional lung tissue develops during prenatal development without communication with the bronchial tree. It is typically supplied by systemic arteries, most commonly from the descending aorta. Pulmonary sequestration is classified into two types: intralobar and extralobar. Common symptoms include respiratory distress, feeding difficulties, recurrent pneumonia, chronic cough, and haemoptysis. Lobectomy remains the treatment of choice; however, in asymptomatic patients, and in the absence of established guidelines, conservative management with periodic monitoring may be considered as a favourable alternative.