Context Middle lobe syndrome (MLS) is a special clinical and radiographic condition that is commonly misdiagnosed in the routine pediatric clinical workup. Delay in the diagnosis may cause irreversible damage of the middle lobe with poor response to medical treatment and may indicate its surgical resection. Aims To evaluate the clinical presentations, diagnostic lines, and surgical management of right MLS in children. Settings and design This is a retrospective descriptive study that involved the records of 26 patients diagnosed as having MLS and referred for surgical resection of middle lobe in the period between March 2012 and May 2019. Patients and methods We reviewed the hospital data of the selected patients in a retrospective manner regarding age, sex, symptoms, duration of these symptoms and the underlying etiology, the findings of chest radiograph, computed tomography, sputum culture, and pathology used for diagnosis. Fiberoptic or rigid bronchoscopy was performed before surgery for diagnostic and/or therapeutic purposes. Standard right middle lobectomy was done in all cases, and postoperative follow-up of clinical symptom improvement and complications was done. Statistical analysis used Graphical representation of the numerical data is present in the form using bar charts. Results We studied 18 (69.2%) females and eight (30.8%) males, with a mean age of 8.09 ±2.9 years. Clinical findings were cough in 76.9% of patients, expectoration in 38.5%, intermittent hemoptysis in 11.5%, chest pain in 19.2%, dyspnea in 34.6%, and 30.7% presented with recurrent or prolonged fever. The mean duration of symptoms was 27.4±16.7 months. Asthma is the most frequent underlying illness (23%). Bronchiectasis of the middle lobe was the most common radiologic (50%) and pathologic finding (42.3%). Tuberculosis was the final pathology in one patient and hydatid cyst was the cause in other one. Surgical resection of the middle lobe was done without reported mortality and with only few minor complications. Conclusion Lobectomy of the right middle lobe is a good choice for management of children with MLS who did not respond to medical treatment with low rate of complications. Keywords: bronchiectasis, children, middle lobe syndrome, surgery