Introduction:Congenital lobar emphysema (CLE) is a rare developmental lung malformation that involves the hyperaeration of one or more lung lobes due to partial obstruction and occurs at a rate of 1/20,000–30,000 live births.Here,we aimed to retrospectively examine the clinical, radiological, and bronchoscopy findings of patients with CLE who were diagnosed and treated with surgical or conservative approaches in our clinic to compare our results with those in the literature. Method:We examined the clinical, and radiological data and FB findings of the patients with CLE aged 0–18 years at our center between 2013 and 2020.We also examined the symptoms and findings recorded during the patients’ follow-up. Results:.The median age of 20 patients with CLE at diagnosis was 3.2 years (range, 1 day–17 years).Respiratory distress and mediastinal shift were more prominent in the patients who underwent surgery than the patients who were followed up conservatively and diagnosed at an early age (p = 0.001, p = 0.049, p = 0.001, respectively). Discussion: In line with studies in the literature, the pulmonary symptoms and CLE-related imaging findings in our study regressed during the conservative follow-up.We recommend clinicians consider performing a detailed anamnesis for patients with unresolved respiratory symptoms and unilateral or bilateral increased ventilation,along with appropriate imaging tests and examinations, and should consider CLE in the diagnosis.