Congenital cystic adenomatoid malformation (CCAM) is a rare congenital cystic abnormality of the lung. Most of cases are usually diagnosed and managed in the newborn period even though some are asymptomatic and present in childhood or adult. The authors report a 7-year-old girl who presented with chronic cough, hemoptysis and clubbing of fingers. Physical examination revealed decreased breath sound and dullness on percussion at right upper chest. A chest radiograph showed a large thin-walled cyst with air fluid and a small thin-walled cyst occupied the whole right upper lobe. Computed tomography (CT) of the chest showed two large thin-walled cavities with air fluid level in the right upper lobe with few small cavities nearby. She was given antibiotics and cardiovascular and thoracic surgeon was consulted. The patient underwent right upper lobectomy. Microscopic examination was compatible with CCAM type 1. The post operative course was uneventful and the recovery was complete. She continued to be healthy at a follow-up visit about 8 months postoperatively but chest radiograph showed soft tissue density at right upper lung field. Chest CT findings were compatible with recurrent CCAM. A follow-up chest radiograph at 13 months postoperatively showed significant reduction in size of the lesion.