Background: Middle Lobe Syndrome (MLS) is a pediatric respiratory condition characterized by the collapse of the middle lobe of the right lung or the lingula of the left lung. Historically linked with tuberculosis, contemporary cases primarily involve asthma and other causes, like foreign body aspiration or mucous plug. Case Presentation: We have, herein, analyzed the clinical features and management of three cases of MLS from a single pediatric center. Additionally, we have reviewed 75 articles on pediatric MLS by using common databases, such as PubMed and Medline. Discussion: MLS often poses a diagnostic challenge, presenting with symptoms, like cough, wheezing, and localized rales. Radiological exams, especially chest X-rays and flexible bronchoscopy, are needed for confirming the diagnosis, particulary in challenging cases. Both obstructive and non-obstructive factors contribute to MLS. Conclusion: A comprehensive understanding of MLS, its diverse causes, and the significance of early diagnosis is crucial for effective management. Treatment should target the underlying cause. Prompt intervention may offer a positive outcome and help prevent complications, like bronchiectasis.