A retrospective analysis of 57 consecutive cases with congenital cystic disease of the lung admitted to King Faisal Specialist Hospital and Research Center and King Khalid University Hospital, Riyadh, between 1985-1995 is presented. There were 37 congenital lobar emphysema (CLE), 7 cystic adenomatoid malformation (CAM), 8 bronchogenic cyst (BC) and 5 pulmonary sequestrations (PS). There were 39 males and 18 females with ages ranging from 1 day to 5 years. All patients were symptomatic except three. Respiratory distress, repeated chest infections, and cystic changes noted in chest x-ray were the commonest presentation. Five of eight patients with BC presented with symptoms related to pressure effect of the cyst on the surrounding structures, these included bronchiectasis in two patients, bronchopleural fistula in one, pulmonary artery stenosis and bronchomalacia in one, airway obstruction mimicking bronchial asthma in one. Seven patients (12.2%) were treated conservatively, the remaining underwent surgery. Surgery included excision of the bronchogenic cyst and lobectomy for CLE, CAM, and intralobar sequestration. The post-operative course in most cases was uneventful. There were no deaths in this series, and the majority of patients had a satisfactory outcome with follow-up ranging from 1-72 months (mean 24 months). It appears that lobectomy for symptomatic CLE, CAM, and intralobar sequestration and excision for bronchogenic cyst offer the best treatment modality and is well tolerated by pediatric patients. Careful search for associated anomalies is important to obtain better outcome.