The "prune belly" syndrome classically described in males, has also been described in female patients with a variable incidence. Associations with multiple respiratory tract malformations have been reported which together with urinary tract anomalies and renal failure are often the cause of early mortality. We report the case of a female patient with prune belly in association with cystic adenomatoid malformation of the lung and pulmonary sequestration with a favorable clinical evolution following medical and surgical treatment.