In four neonates, two boys and two girls, congenital cystic malformation of the lung was diagnosed. Two cases were diagnosed during prenatal ultrasound investigation. One of the patients recovered by a wait-and-see policy, one after antibiotic treatment and two following surgical removal of the diseased lung sections. Congenital pulmonary cysts are rare; congenital cystadenomatoid malformation (CCAM) is the most prevalent form. The most common presentation of CCAM is respiratory distress immediately following birth. Some children are asymptomatic at birth, but present with recurrent respiratory infections later in childhood. With the increasing use of antenatal ultrasound, an increasing number of cystic lung lesions are diagnosed antenatally. The prognosis of these mostly asymptomatic lesions is not clear and indications for prenatal and postnatal interventions must be determined by a multidisciplinary approach. Recently, malignant pulmonary tumours were found to be associated with CCAM. This finding necessitates long-term follow-up, even of asymptomatic CCAM lesions.

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