Congenital malformations of the lung are rare and vary widely in their presentation and severity. However, frequently they present with dramatic symptoms resulting in the need for prompt diagnosis and treatment. Five patients with congenital lung malformations were treated in Karadeniz Technical University Farabi Medical School Pediatric Surgery Department between June 1992 and January 1997. There were one girl and four boys. The mean age was 42 days (range 1 to 120 days) and the mean weight was 3.9 kg (range 2.4 to 5.4 kg). The following cases were included; one pulmonary agenesis, two congenital lobar emphysema, one pulmonary sequestration, and one cystic adenomatoid malformation. Respiratory distress was the most common presenting symptom. The diagnosis was performed by chest X-ray with postero-anterior and lateral view and thoracic computed tomography (CT). Associated congenital anomalies were detected in one patient and the following anomalies were found; esophageal atresia, tracheo-esophageal fistula, short distal esophagus, and small intrathoracic stomach Posterolateral thoracotomy was used for all patients and complete surgical resection was performed in four patients. Corrective surgery was delayed in one patient in whom extended respiratory support was required and he died on the ninth day due to respiratory failure. In four patients prognosis was excellent. In this article, diagnosis and treatment of congenital lung malformations are discussed.