Objective: Right middle lobe syndrome is a rare entity in children, causing high morbidity. Our experience of these patients including their clinical and laboratory characteristics, indications forsurgical management, postoperative courses, and follow-up results was evaluated. Methods: A retrospective analysis was performed involving 20 children with right middle lobe syndrome who were hospitalized and treated with surgical resection of the right middle lobe in Van Training and Research Hospital and Yuzuncuyil university hospital, Turkey, between January 2002 and January 2011. Results: The mean age of the patients was 10.5 years (range, 5 to 15 years). Twelve patients were boys and eight were girls. The most frequent symptom was chronic cough (75). Hemoptysis was present in two (10) patients. One patient was being treated for asthma. 25 positive cultures were identified among the patients. Streptococcus pneumoniae was the most frequently identified agent in sputum. All patients underwent chest computed tomography. There were bronchiectasis in 11 (55) patients, atelectasis and bronchiectasis in five (25) patients, and destroyed lung in four (20) patients. A narrowed middle lobe bronchus was shown in 15 (75) patients. Bronchoscopy was performed in 18 (90) patients. Stenosis due to external compression was seen in 12 (60) patients, hyperemia and bronchitis in two (10) patients, granulation tissue in two (10) patients, and dense secretions in two (10) patients. A history of doctor-diagnosed tuberculosis was present in two (10) patients. These patients had completed antituberculous treatment. The patients had been symptomatic for the last 1 to 10 years (mean, 4 years) and had received several medical treatments. All patients (totally 20 patients) underwent right middle lobe resection. In one patient, a bronchial abnormality was found intraoperatively. One patient died on postoperative day 10 due to a brain abscess. Three other patients had postoperative complications (15). Mean duration of follow-up of the patients was 4.5 years (range, 2 months to 12 years). Seventeen patients were asymptomatic, and two patients had improved. Conclusions: Children with right middle lobe syndrome unresponsive to medical treatment should undergo early lobe resection to avoid serious complications and the progression of the disease to other segments or lobes.